Wilcox's Surgical Anatomy of The Heart 4th Ed (PDF) (Tahir99) VRG PDF

Wilcoxs Surgical Anatomy
of the Heart
Fourth edition
Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:08:17 WEST 2013.
http://ebooks.cambridge.org/ebook.jsf?bid=CBO9781139028561
Cambridge Books Online Cambridge University Press, 2013
Wilcoxs Surgical Anatomy

of the Heart
Fourth edition
Robert H. Anderson, BSc, MD, FRCPath
Visiting Professor, Institute of Genetic Medicine, Newcastle University, Newcastle-upon-Tyne, UK;
Visiting Professor of Pediatrics, Medical University of South Carolina, Charleston, SC, USA
Diane E. Spicer, BS, PA(ASCP)

Pathologists Assistant, University of Florida Pediatric Cardiology, Gainesville, Florida, and
Congenital Heart Institute of Florida, St. Petersburg, FL, USA
Anthony M. Hlavacek, MD
Associate Professor, Department of Pediatrics, Medical University of South Carolina, Charleston, SC, USA
Andrew C. Cook, BSc, PhD

Senior Lecturer, Cardiac Unit, Institute of Child Health, University College London, London, UK
Carl L. Backer, MD
A. C. Buehler Professor of Surgery, Northwestern University Feinberg School of Medicine, Ann & Robert H. Lurie
Childrens Hospital of Chicago, Chicago, IL, USA
University Printing House, Cambridge CB2 8BS, United Kingdom

Cambridge University Press is part of the University of Cambridge.
It furthers the Universitys mission by disseminating knowledge in the pursuit of
education, learning, and research at the highest international levels of excellence.
www.cambridge.org
Information on this title: www.cambridge.org/9781107014480
Fourth edition Robert H. Anderson, Diane E. Spicer, Anthony M. Hlavacek, Andrew C. Cook, and Carl L. Backer 2013
This publication is in copyright. Subject to statutory exception
and to the provisions of relevant collective licensing agreements,
no reproduction of any part may take place without the written
permission of Cambridge University Press.
Fourth edition rst published 2013
Third edition rst published 2004
Printed and bound by Grafos SA, Arte sobre papel, Barcelona, Spain
A catalogue record for this publication is available from the British Library
Library of Congress Cataloguing in Publication data
Anderson, Robert H. (Robert Henry), 1942
Wilcoxs surgical anatomy of the heart. Fourth edition / Robert H. Anderson, BSc, MD, FRCPath, Diane E. Spicer,
BS, Anthony M. Hlavacek, MD, Andrew C. Cook, BSc, PhD, Carl L. Backer, MD.
pages cm
ISBN 978-1-107-01448-0 (hardback)
1. Heart Anatomy. 2. Heart Surgery. I. Title. II. Title: Wilcoxs Surgical Anatomy of the Heart.
QM181.W55 2013
6110 .12dc23
2012051614
ISBN 978-1-107-01448-0 Hardback
Cambridge University Press has no responsibility for the persistence or accuracy of
URLs for external or third-party internet websites referred to in this publication,
and does not guarantee that any content on such websites is, or will remain,
accurate or appropriate.
Every effort has been made in preparing this book to provide accurate and
up-to-date information which is in accord with accepted standards and practice
at the time of publication. Although case histories are drawn from actual cases,
every effort has been made to disguise the identities of the individuals involved.
Nevertheless, the authors, editors and publishers can make no warranties that the
information contained herein is totally free from error, not least because clinical
standards are constantly changing through research and regulation. The authors,
editors and publishers therefore disclaim all liability for direct or consequential
damages resulting from the use of material contained in this book. Readers
are strongly advised to pay careful attention to information provided by the
manufacturer of any drugs or equipment that they plan to use.
Contents
Preface
page vii
Acknowledgements
viii
Surgical approaches to the heart
Anatomy of the cardiac chambers
13
Surgical anatomy of the valves of the heart
51
Surgical anatomy of the coronary circulation
90
Surgical anatomy of the conduction system
111
Analytical description of congenitally malformed hearts
128
Lesions with normal segmental connections
150
Lesions in hearts with abnormal segmental connections
244
Abnormalities of the great vessels
321
Positional anomalies of the heart
363
Index
377
Preface
The books and articles devoted to

technique in cardiac surgery are legion.
This is most appropriate, as the success of
cardiac surgery is greatly dependent upon
excellent operative technique. But
excellence of technique can be dissipated
without a rm knowledge of the underlying
cardiac morphology. This is just as true for
the normal heart as for those hearts with
complex congenital lesions. It is the
feasibility of operating upon such complex
malformations that has highlighted the
need for a more detailed understanding of
the basic anatomy in itself. Thus, in recent
years surgeons have come to appreciate the
necessity of avoiding damage to the
coronary vessels, often invisible when
working within the cardiac chambers, and
particularly to avoid the vital conduction
tissues, invisible at all times. Although
detailed and accurate descriptions of the
conduction system have been available
since the time of their discovery, only
rarely has its position been described with
the cardiac surgeon in mind. At the time
the rst edition of this volume was
published, to the best of our knowledge
there had been no other books that
specically displayed the anatomy of
normal and abnormal hearts as perceived at
the time of operation. We tried to satisfy
this need in the rst volume by combining
the experience of a practising cardiac
surgeon with that of a professional cardiac
anatomist. We added signicantly to the
illustrations in the second edition, while
seeking to retain the overall concept, as
feedback from those who had used the rst

edition was very positive. In the third
edition, we sought to expand and improve
still further on the changes made in the
second edition. In the second edition, we
had added an entirely new chapter on
cardiac valvar anatomy, and greatly
expanded our treatment of coronary
vascular anatomy. We retained this format
in the third edition, as we were gratied
that, as hoped, readers were able to nd a
particular subject more easily. The third
edition also contained still more new
illustrations, retaining the approach of
orientating these illustrations, where
appropriate, as seen by the surgeon
working in the operating room, but
reverting to anatomical orientation for most
of the pictures of specimens. So as to clarify
the various orientations of each individual
illustration, we continued to include a set of
axes showing, when appropriate, the
directions of superior, inferior, anterior,
posterior, left, right, apex, and base. All
accounts were based on the anatomy as it is
observed and, except in the case of
malformations involving the aortic arch and
its branches, they owe nothing to
speculative embryology.
A major change was forced upon us as
we prepared this fourth edition, as our
original surgical author, Benson Wilcox,
died in May of 2010. It is very difcult to
replace such a pioneer and champion of
surgical education, but we are gratied that
Carl Backer has assumed the role of
surgical editor. We are also pleased to add
Diane Spicer to our anatomical team. She

has contributed enormously by providing
many new and better illustrations of the
anatomy as seen in the autopsied heart.
These advances are complimented by the
contributions of our other new editor,
Tony Hlavacek. Tony has provided quite
remarkable images obtained using
computed tomography and magnetic
resonance imaging, which show that the
heart can be imaged with just as much
accuracy during life as when we hold the
specimens in our hands on the autopsy
bench. Recognising the huge contributions
of Ben Wilcox, we are also pleased to
rename this fourth edition Wilcoxs
Surgical Anatomy of the Heart. As with
the previous editions, it is our hope that the
new edition will continue to be of interest
not only to the surgeon, but also to the
cardiologist, anaesthesiologist, and surgical
pathologist. All of these practitioners
ideally should have some knowledge of
cardiac structures and their exquisite
intricacies, particularly those cardiologists
who increasingly treat lesions that
previously were the province of the surgeon.
Our senior anatomist remains active, and
has been fortunate to be granted access to
several archives of autopsied hearts held in
the United States of America subsequent to
his retirement from the Institute of Child
Health in London. We remain condent
that, in the hands of this new team, and if
supply demands, the book will pass through
still further editions, hopefully continuing
to improve with each version.
Robert H. Anderson, Diane E. Spicer,
Anthony M. Hlavacek,
Andrew C. Cook,
and Carl L. Backer,
London, Tampa, Charleston and Chicago
November, 2012
http://dx.doi.org/10.1017/CBO9781139028561.001
Acknowledgements
A good deal of the material displayed in
these pages, and the concepts espoused, are
due in no small part to the help of our
friends and collaborators. As indicated in
our preface, the major change since we
produced the third edition has been the sad
passing of our founding surgical editor,
Benson R. Wilcox. We have renamed this
fourth edition Wilcoxs Surgical Anatomy
of the Heart. We dedicate this edition to
his eternal memory. A further change has
been the retirement of Robert H. Anderson
from the Institute of Child Health at
Great Ormond Street Childrens Hospital,
London. Retirement, however, has
permitted him to establish new
connections, not least with the newest
additions to our team of authors. This has
permitted many new hearts to be
specically photographed for this new
edition, not only of autopsy specimens, but
also in the operating room. In addition, it
has created the collaboration that permits
the inclusion of wonderful images
obtained using computed tomography and

magnetic resonance imaging. We
continue, nonetheless, to owe a particular
debt to Anton Becker of the University of
Amsterdam, Bob Zuberbuhler of
Childrens Hospital of Pittsburgh,
Pennsylvania, United States of America,
and F. Jay Fricker of University of Florida,
Gainesville, Florida, United States of
America, all of whom permitted us to use
material from the extensive collections of
normal and pathological specimens held in
their centres. We also continue to
acknowledge the debt owed to Siew Yen
Ho, of the National Heart and Lung
Institute, part of Imperial College in
London. Yen produced many of the
original drawings from which we
prepared our artwork, and photographed
many of the hearts in the Brompton
archive. The initial photographs and
surgical artwork could not have been
produced without the considerable help
given by the Department of Medical
Illustrations and Photography, University

of North Carolina. As with the third
edition, we owe an equal debt of gratitude
to Gemma Price, who has continued to
improve our series of cartoons. For both
the third edition and this edition, she has
worked over and above the call of duty. We
also thank Vi Hue Tran, who helped
photograph the hearts from Great
Ormond Street. We are again indebted to
Christine Anderson for her help during
the preparation of the manuscript, and
thank the team supporting Carl Backer at
Lurie Childrens of Chicago, in
particular Pat Heraty and Anne E. Sarwark.
Finally, it is a pleasure to acknowledge the
support provided by Cambridge
University Press, who have ensured that
all the good parts of the previous
editions were retained. In particular, we
thank Nicholas Dunton and Joanna
Chamberlin for all their help
during the preparation of the book for
publication.
Surgical approaches
to the heart
Wilcoxs Surgical Anatomy of the Heart
When we describe the heart in this chapter,

and in subsequent chapters, our account
will be based on the organ as viewed in its
anatomical position1. Where appropriate,
the heart will be illustrated as it would be
viewed by the surgeon during an operative
procedure, irrespective of whether the
pictures are taken in the operating room, or
are photographs of autopsied hearts. When
we show an illustration in non-surgical
orientation, this will be clearly stated.
In the normal individual, the heart lies
in the mediastinum, with two-thirds of its
bulk to the left of the midline (Figure 1.1).
The surgeon can approach the heart, and
the great vessels, either laterally through
the thoracic cavity, or directly through the
mediastinum anteriorly. To make such
approaches safely, knowledge is required of
the salient anatomical features of the chest
wall, and of the vessels and the nerves that
course through the mediastinum
(Figure 1.2). The approach used most
frequently is a complete median
sternotomy, although increasingly the
trend is to use more limited incisions. The
incision in the soft tissues is made in the
midline between the suprasternal notch
and the xiphoid process. Inferiorly, the
white line, or linea alba, is incised between

the two rectus sheaths, taking care to avoid
entry to the peritoneal cavity, or damage to
an enlarged liver, if present. Reection of
the origin of the rectus muscles in this area
reveals the xiphoid process, which is
incised to provide inferior access to the
anterior mediastinum. Superiorly, a
vertical incision is made between the
sternal insertions of the
sternocleidomastoid muscles. This exposes
the relatively bloodless midline raphe
between the right and left sternohyoid and
sternothyroid muscles. An incision
through this raphe gives access to the
superior aspect of the anterior
mediastinum. The anterior mediastinum
immediately behind the sternum is devoid
of vital structures, so that the superior and
inferior incisions into the mediastinum can
safely be joined by blunt dissection in the
retrosternal space. Having split the
sternum, retraction will reveal the
pericardial sac, lying between the pleural
cavities. Superiorly, the thymus gland
wraps itself over the anterior and lateral
aspects of the pericardium in the area of
exit of the great arteries, the gland being a
particularly prominent structure in the
infant (Figures 1.3, 1.4). It has two lateral

lobes, joined more or less in the midline.
Sometimes this junction between the lobes
must be divided, or partially excised, to
provide adequate exposure. The arterial
supply to the thymus is from the internal
thoracic and inferior thyroid arteries. If
divided, these arteries tend to retreat into
the surrounding soft tissues, and can
produce troublesome bleeding. The veins
draining the thymus are fragile, often
emptying into the left brachiocephalic or
innominate vein via a common trunk
(Figure 1.5). Undue traction on the gland
can lead to damage to this major vessel.
When the pericardial sac is exposed
within the mediastinum, the surgeon
should have no problems in gaining access
to the heart. The vagus and phrenic nerves
traverse the length of the pericardium, but
are well lateral (Figures 1.2, 1.6). The
phrenic nerve on each side passes
anteriorly, and the vagus nerve posteriorly,
relative to the hilum of the lung
(Figure 1.6).
At operation, the course of the phrenic
nerve is seen most readily through a lateral
thoracotomy (Figure 1.7). It is when the
heart is approached through a median
Long axis of body

Obtuse margin
Long axis of heart
Fig. 1.1 The computed tomogram, with the
Acute margin
Apex
cardiac cavities delimited subsequent to

injection of contrast material, shows the
relationships of the heart to the thoracic
structures well. Note the discordance between
the cardiac long axis and the long axis of the
body.
Thymic veins
Sup.
Brachiocephalic vein
Left
Right
Right phrenic
nerve
Inf.
Superior
caval vein
Left phrenic
nerve
Pulmonary
trunk
Aorta
Left atrial
appendage
Right atrium
Fig. 1.2 This view, taken at autopsy,

Right ventricle
Left ventricle
sternotomy, therefore, with the nerve not

immediately evident, that it is most liable to
injury. Although it can sometimes be seen
through the reected pericardium
(Figure 1.8), its proximity to the superior
caval vein (Figures 1.2, 1.9, 1.10), or to a
persistent left caval vein when that
structure is present (Figure 1.11), is not
always easily appreciated when these
vessels are dissected from the anterior
approach. Near the thoracic inlet, it passes
close to the internal thoracic artery
(Figures 1.6, 1.10), exposing it to injury
either directly during takedown of that
vessel, or by avulsing the pericardiophrenic
artery with excessive traction on the chest
wall. The internal thoracic arteries
themselves are most vulnerable to injury
during closure of the sternum. The phrenic
nerve may be injured when removing the
pericardium to use as a cardiac patch, or
when performing a pericardiectomy.
Injudicious use of cooling agents within the
pericardial cavity may also lead to phrenic

paralysis or paresis.
A standard lateral thoracotomy provides
access to the heart and great vessels via the
pleural space. Left-sided incisions provide
ready access to the great arteries, left
pulmonary veins, and the chambers of the
left side of the heart. Most frequently, the
incision is made in the fourth intercostal
space. The posterior extent is through the
triangular, and relatively bloodless, space
between the edges of the latissimus dorsi,
trapezius, and teres major muscles
(Figure 1.12). The oor of this triangle is
the sixth intercostal space. Division of the
latissimus dorsi, and a portion of trapezius
posteriorly, frees the scapula so that the
fourth intercostal space can be identied.
Its precise identity should be conrmed by
counting down the ribs from above. The
so-called muscle sparing thoracotomy is
designed to preserve the latissimus dorsi
and serratus anterior muscles. In cases
demonstrates the anatomical relationships of

the vessels and nerves within the mediastinum.
requiring greater degrees of exposure, the

latissimus dorsi can be partially divided. It
is rarely necessary, if ever, to divide the
serratus anterior. The intercostal muscles
are then divided equidistant between the
fourth and fth ribs. The incision is rarely
carried forward beyond the midclavicular
line in a submammary position, and care is
taken to avoid damage to the nipple and the
tissue of the breast. The intercostal
neurovascular bundle is well protected
beneath the lower margin of the fourth rib.
Having divided the musculature as far as
the pleura, the pleural space is entered, and
the lung permitted to collapse away from
the chest wall. Posterior retraction of the
lung reveals the middle mediastinum, in
which the left lateral lobe of the thymus,
with its associated nerves and vessels, is
seen overlying the pericardial sac and the
aortic arch. Intrapericardial access is
usually gained anterior to the phrenic
nerve. On occasion, the thymus gland may
Sup.
Right
Left
Inf.
Thymus
Pericardial sac
Diaphragm
Fig. 1.3 This view, taken at autopsy,

demonstrates the extent of the thymus as it
extends over the anterior and lateral aspects of
the pericardial sac at the base of the heart.
Note the haemorrhagic pericardial effusion.
Left
Left lobe
of thymus
Sup.
Inf.
Right
Pericardial
sac
Right lobe
of thymus
Fig. 1.4 This view, taken in the operating

Phrenic nerve
Superior caval vein
require elevation when the incision is

extended superiorly, precautions being
taken to avoid unwanted damage as
discussed earlier. The lung is retracted
anteriorly to approach the aortic isthmus
and descending thoracic aorta, and the

parietal pleura is divided on its mediastinal
aspect. This is usually done posterior to the
vagus nerve. In this area, the vagus nerve
gives off its left recurrent laryngeal branch,
room through a median sternotomy in an

infant, shows the extent of the thymus gland.
Note the right phrenic nerve adjacent to the
superior caval vein.
which passes around the inferior border of

the arterial ligament, or the duct if the
arterial channel is still patent (Figure 1.13).
The recurrent nerve then ascends towards
the larynx on the medial aspect of the
Left
Sup.
Inf.
Right
Thymic
veins
Aorta in
pericardium
Right lobe of
thymus
Fig. 1.5 This operative view, again taken through a median

sternotomy, shows the delicate veins that drain from the thymus
gland to the left brachiocephalic veins.
Left pericardiophrenic
artery and vein
Left phrenic nerve
Left vagus & recurrent laryngeal nerve
Left internal thoracic artery

Right phrenic nerve
Right vagus & recurrent laryngeal nerve
Right pericardiophrenic
artery and vein
Fig. 1.6 As shown in this cartoon of a median

sternotomy, the pericardium can be opened in
the midline so that the phrenic and vagus
nerves stay well clear of the operating eld.
Ant.
Inf.
Sup.
Post.
Left pericardiophrenic vein
Left phrenic nerve

Left pericardiophrenic artery
Fig. 1.7 This operative view, taken through a

left lateral thoracotomy, shows the course of
the left phrenic nerve over the pericardium.
Left
Right atrial
appendage
Sup.
Inf.
Right
Superior caval
vein
Right phrenic nerve

median sternotomy, shows the right phrenic
nerve as seen through the reected
pericardium.
posterior wall of the aorta, running adjacent

to the oesophagus. Excessive traction of the
vagus nerve as it courses into the thorax
along the left subclavian artery can cause
injury to the recurrent laryngeal nerve just
as readily as can direct trauma to the nerve
in the environs of the ligament. The
superior intercostal vein is seen crossing
the aorta, then insinuating itself between
the phrenic and vagus nerves (Figures 1.11,

1.14, 1.15). This structure, however, is
rarely of surgical signicance, but is
frequently divided to provide surgical
access to the aorta. The thoracic duct
(Figure 1.16) ascends through this area,
draining into the junction of the left
subclavian and internal jugular veins.
Accessory lymph channels draining into
the duct, which is usually posteriorly

located and runs along the vertebral
column, can be troublesome when
dissecting the origin of the left subclavian
artery.
A right thoracotomy, in either the
fourth or fth interspace, is made through
an incision similar to that for a left one. The
fth interspace is used when approaching
Cut edge of
pericardium
Left
Right phrenic
nerve
Sup.
Inf.
Right

median sternotomy having pulled back the
edge of the pericardial sac, shows the right
phrenic nerve in relation to the right
pulmonary veins.
Right pulmonary veins
Right internal
thoracic artery
Right phrenic nerve
Superior
caval vein
Ant.
Inf.
Sup.
Azygos vein
Post.
Fig. 1.10 This operative view, taken through

a right thoracotomy, shows the relationship of
the right phrenic nerve to the right internal
thoracic artery and the superior caval vein.
Left phrenic nerve

Superior
intercostal vein
Arch of aorta
Ant.
Persistent left
superior caval vein
Inf.
Sup.
Fig. 1.11 This operative view, taken through a left thoracotomy, shows the
relationship of the left phrenic nerve to a persistent left superior caval vein.
Note also the course of the superior intercostal vein.
Post.
Teres major
Latissimus dorsi
Trapezius
Bloodless triangle
Fig. 1.12 The cartoon shows the location of

the bloodless area overlying the posterior
extent of the sixth intercostal space.
Ant.
Left vagus nerve
Inf.
Sup.
Post.
Patent arterial
duct
Left recurrent
laryngeal nerve
Fig. 1.13 This operative view, taken through a left lateral thoracotomy in an
adult, shows the left recurrent laryngeal nerve passing around the arterial duct.
Left vagus nerve
Superior
intercostal
vein
Superior
caval vein
Sup.
Ant.
Arterial duct
Post.
Inf.
Fig. 1.14 The anatomical image shows the course of the left superior
intercostal vein.
10
Left phrenic nerve

Left vagus
nerve
Left superior
intercostal vein
Aorta
Left subclavian
artery
Ant.
Inf.
Sup.
Fig. 1.15 This operative view, taken through a left lateral thoracotomy,
Post.
Aortic isthmus
shows the course of the left superior intercostal vein. (Compare with
Figure 1.14.)
Left subclavian artery
Ant.
Thoracic
duct
Inf.
Sup.
Fig. 1.16 In this operative view, taken through a left

Post.
thoracotomy, the thoracic duct is seen coursing below the left

subclavian artery to its termination in the brachiocephalic vein.
Left
Superior
caval vein
Sup.
Left brachiocephalic
vein
Inf.
Right
Fig. 1.17 This anatomical image, taken at

Trachea
Azygos vein
Right common
carotid artery
autopsy, shows the normal location of the

azygos vein as it extends along the spine,
receives the intercostal veins, and crosses over
the root of the right lung to empty into the
Intercostal veins
Brachiocephalic artery
Right recurrent
laryngeal nerve
Left
Sup.
Inf.
Right subclavian artery

Right
Fig. 1.18 This operative view, taken through

a median sternotomy, shows the course of the
right recurrent laryngeal nerve relative to the
right subclavian artery.
11
12
the heart, while the fourth permits access to

the right-sided great vessels. Access to the
pericardium is gained by incising anterior
to the phrenic nerve, this approach often
necessitating retraction of the right lobe of
the thymus. To reach the right pulmonary
artery, and its adjacent mediastinal
structures, it is sometimes useful to divide
the azygos vein near its junction with the
superior caval vein (Figure 1.17).
Extension of this incision superiorly
exposes the origin of the right subclavian
branch of the brachiocephalic trunk.
Laterally, this artery is crossed by the right
vagus nerve, the right recurrent laryngeal

nerve taking origin from the vagus and
curling around the posteroinferior wall of
the artery before ascending into the neck
(Figure 1.18). Also encircling the
subclavian origin on this right side is the
subclavian sympathetic loop, the so-called
ansa subclavia, a branch of the sympathetic
trunk that runs up into the neck. Damage
to this structure can produce Horners
syndrome.
An anterior right or left thoracotomy is
occasionally used in treating congenital
malformations. Once the chest is opened,
the same basic anatomical rules apply as

described previously. Thus far, our
account has presumed the presence of
normal anatomy. In many instances, the
disposition of the thoracic structures will
be altered by a congenital malformation.
These alterations will be described in the
appropriate sections.
Reference
1. Cook AC, Anderson RH. Attitudinally
correct nomenclature. Heart 2002; 87:
503506.
Anatomy of the
cardiac chambers
14
Regardless of the surgical approach, once

having entered the mediastinum, the
surgeon will be confronted by the heart
enclosed in its pericardial sac. In the strict
anatomical sense, this sac has two layers,
one brous and the other serous. From a
practical point of view, the pericardium is
essentially the tough brous layer; the
serous component forms the lining of the
brous sac, and is reected back onto the
surface of the heart as the epicardium. It is
the brous sac, therefore, which encloses
the mass of the heart. By virtue of its own
attachments to the diaphragm, it helps
support the heart within the mediastinum.

Free-standing around the atrial chambers
and the ventricles, the sac becomes
adherent to the adventitial coverings of
the great arteries and veins at their
entrances to and exits from it, these
attachments closing the pericardial cavity.
The cavity of the pericardium is limited
by the two layers of serous pericardium,
which are folded on one another to
produce a double-layered arrangement.
The outer or parietal layer is densely
adherent to the brous pericardium, while
the inner layer is rmly attached to the
myocardium, and is the epicardium

(Figure 2.1). The pericardial cavity,
therefore, is the space between the inner
parietal serous lining of the brous
pericardium and the surface of the heart
(Figure 2.2). There are two recesses
within the cavity that are lined by serous
pericardium. The rst is the transverse
sinus, which occupies the inner curvature
of the heart (Figure 2.3). Anteriorly, it is
bounded by the posterior surface of the
great arteries. Posteriorly, it is limited by
the right pulmonary artery and the roof of
the left atrium. There is a further recess
Aorta
Transverse sinus
Right pulmonary
artery
Pericardial cavity
Left atrium
Oblique sinus
Ant.
Base
Apex
Post.
Visceral
Parietal
Fibrous
pericardium
Serous pericardium
Fig. 2.1 The cartoon shows the arrangement of the

pericardial cavity as seen in a parasternal long axis view.
Left
Left appendage
Inf.
Sup.
Right
*
*
*
*
*
Pulmonary
trunk
Right ventricle
Aorta
*
*
Right
appendage
Fibrous
pericardium
*
*
Pericardial cavity
Fig. 2.2 The operative view through a

median sternotomy shows the anterior surface
of the heart following a pericardial incision.
The white asterisks show the extent of the
pericardial cavity.
from the transverse sinus that extends

between the superior caval and the right
upper pulmonary veins, with its right
lateral border being a pericardial fold
between these vessels (Figure 2.4). When
exposing the mitral valve through a left
atriotomy, incisions through this fold,
along with mobilisation of the superior
caval vein, provide excellent access to the

superior aspect of the left atrium and the
right pulmonary artery. This fold is also
incised when a snare is placed around the
superior caval vein. Laterally, on each
side, the ends of the transverse sinus are in
free communication with the remainder of
the pericardial cavity.
15
The second pericardial recess is the

oblique sinus. This is a blind-ending cavity
behind the left atrium (Figure 2.5), with its
upper boundary formed by the reection of
serous pericardium between the upper
pulmonary veins. The right border is the
reection of pericardium around the right
pulmonary veins and the inferior caval
Left
Sup.
Pulmonary trunk
Inf.
Right
Aorta
Right
appendage
Clamp in transverse
sinus
Fig. 2.3 Operative view through a median

sternotomy. The clamp has been passed
through the transverse sinus.
Left
Clamp tenting pericardial fold
Inf.
Sup.
Right
Fig. 2.4 Operative view through a median
Superior caval vein
sternotomy showing the posterior recess of the

transverse sinus limited by a pericardial fold
around the superior caval vein. In this picture,
the fold is being tented by a right-angled clamp
passed behind the superior caval vein.
16
vein, while the left border is the reection

of pericardium around the left pulmonary
veins (Figure 2.6).
With the usual surgical approach
through a median sternotomy, the brous
pericardium is opened more-or-less in the
Oblique ligament
Pulmonary trunk
midline and retracted laterally, exposing

the anterior sternocostal surface of the heart
and great vessels. The pulmonary trunk and
aorta are seen leaving the base of the heart
and extending in a superior direction, with
the aortic root in the posterior and
rightward position (Figure 2.2). Should

the aortic root not be in this expected
relationship, the ventriculoarterial
connections will almost always be abnormal
(see Chapter 8). The atrial appendages are
usually seen one to either side of the
Left pulmonary veins
Left atrial
appendage
Sup.
Right
Left
Fig. 2.5 Anatomical view showing the oblique sinus of the

Oblique sinus
Inf.
pericardial cavity, which lies behind the left atrium. Note the
oblique ligament, which occupies the site during development of
the left superior caval vein.
Sup.
Coronary sinus
Right atrium
Right
Left
Inf.
Inferior caval vein
Oblique sinus
Fig. 2.6 The heart has been reected superiorly from its
pericardial cradle to show the location of the oblique sinus.
prominent arterial pedicle. The

morphologically right appendage is more
prominent. It has a blunt triangular shape,
and possesses a broad junction with the
atrial cavity (Figure 2.7). The
morphologically left appendage may not be
seen immediately. When found at the left
border of the pulmonary trunk, it is a
tubular structure, having a narrow junction
with the rest of the atrium (Figure 2.8). The
presence of the two appendages on the same
side of the arterial pedicle is an anomaly in

itself, which is called juxtaposition. This
arrangement is most often associated with
additional malformations within the heart
(see Chapter 8). Inspection of the left border
of the heart should always include a search
for persistence of the left superior caval
vein. When present, the venous channel
will be found by following the course of the
left pulmonary artery. The vein crosses
anterior to the pulmonary artery and is seen
superiorly within the pericardial cavity,

with the left atrial appendage located
anteriorly and laterally (Figure 2.9).
Within the pericardial cavity, it extends
down the posterior aspect of the left atrium,
passing through the inferior left
atrioventricular groove to reach the right
atrial orice of the coronary sinus
(Figure 2.10).
The ventricular mass extends from the
atrioventricular grooves to the apex, and
Left
Sup.
Inf.
Right
Right ventricle
Aorta
Right atrial
appendage
Fig. 2.7 Operative view through a median sternotomy showing

the typical triangular shape of the morphologically right atrial
appendage.
Left atrial appendage
Left
Sup.
17
Inf.
Right
Pulmonary trunk
Subpulmonary infundibulum

the tubular morphologically left atrial appendage.
18
usually extends into the left hemithorax.

An anomalous position of the ventricular
mass, or its apex, is again highly suggestive
of the presence of congenital cardiac
malformations (see Chapter 10). In shape,

the ventricular mass is a three-sided
pyramid, having inferior diaphragmatic,
anterior sternocostal, and posterior
pulmonary surfaces (Figure 2.11). The

margin between the rst two surfaces is
sharp. Because of this, it is described as the
acute margin. The angulations of the
Pulmonary trunk
Left
Sup.
Inf.
Fig. 2.9 The operative view through a median sternotomy shows

the location of a persistent left superior caval vein, snared by the
surgeon in this image.
Right
Left superior caval vein

Left atrial
appendage
Sup.
Post.
Fig. 2.10 The base of the heart has been dissected by removing
Ant.
Inf.
Coronary sinus
the atrial walls. The dissection shows the course of a persistent left
superior caval vein as it passes through the left atrioventricular
groove (red dotted lines), emptying into the right atrium through
the enlarged orice of the coronary sinus.
Sup.
19
Obtuse margin
Post
Ant
Inf.
Sternocostal
Pulmonary
Diaphragmatic
Acute margin
Fig. 2.11 The heart has been removed from the chest and is
viewed from its apex, showing the surfaces of the ventricular cone.
margins between the pulmonary and the

sternocostal surfaces anteriorly, and the
pulmonary and diaphragmatic surfaces
posteriorly, are much more obtuse. The
surgeon encounters these obtuse marginal
areas when the apex of the heart is tipped
out of the pericardium. They are supplied
by the obtuse marginal branches of the
circumex coronary artery. The greater
part of the anterior surface of the
ventricular mass is occupied by the
morphologically right ventricle, with its left
border marked by the anterior
interventricular or descending branch of
the left coronary artery. This artery curves
onto the ventricular surface between the
left atrial appendage and the basal origin of
the pulmonary trunk. The right border of
the morphologically right ventricle is
marked by the right coronary artery, which
runs obliquely in the atrioventricular
groove. Unusually prominent coronary
arteries coursing on the ventricular surface
should always raise the suspicion of
signicant cardiac malformations.
The surface anatomy of the heart is
helpful in determining the most appropriate
site for an incision to gain access to a given
cardiac chamber. For example, the relatively
bloodless outlet portion of the right ventricle
just beneath the origin of the pulmonary
trunk affords ready access to the cavity of
the subpulmonary infundibulum
(Figure 2.8). The important landmark for
the right atrium is the terminal groove, or

sulcus terminalis. This marks the junction
between the appendage and the systemic
venous component of the right atrium
(Figure 2.12). The sinus node is located
within this groove, usually laterally and
inferiorly relative to the superior cavoatrial
junction (Figures 2.13, 2.14), but
occasionally extending over the crest of the
appendage (Figure 2.15). The clinically
signicant artery to the sinus node can also
be seen on occasion, either as it crosses the
crest of the right appendage, or as it courses
behind the superior caval vein to enter the
terminal groove between the orices of the
caval veins. Posterior to, and parallel with,
the terminal groove is a second, deeper
groove, which interposes between the cavity
of the right atrium and the right pulmonary
veins. The surgeon can use this interatrial
groove, known as Waterstons or
Sondergaards groove, to gain access to the
left atrium (Figure 2.16), either by making
an incision in the oor of the groove, or
through the left atrial roof. The latter area is
seen behind the aorta, to the left of the
superior cavoatrial junction (Figure 2.12).
MORPHOLOGICALLY RIGHT
ATRIUM
The right atrium has three basic parts, the
appendage, the venous component
receiving the systemic venous return, and
the vestibule of the tricuspid valve. It also

has a small body, but the boundaries of this
part usually cannot be distinguished from
the venous sinus. It is separated from the
left atrium by the septum. The junction of
the appendage and the systemic venous
sinus is identied externally by the
prominent terminal groove (Figure 2.12).
Internally, the groove corresponds with the
terminal crest, which gives origin to the
pectinate muscles of the appendage
(Figure 2.17). In shape, the appendage is
blunt and triangular, having a wide
junction to the venous sinus across the
terminal groove. The venous sinus is much
smaller when viewed externally, with only
that part extending between the terminal
and Waterstons grooves being visible to
the surgeon. It receives the superior and
inferior caval veins at its extremities.
Superiorly and anteriorly, the appendage
has a particularly important relation with
the superior caval vein. Here, the
appendage terminates in a prominent crest
(Figure 2.13). This forms the summit of
the terminal groove, and is continuous in
the transverse sinus behind the aorta with
the interatrial groove (Figure 2.14).
Absence of such a right-sided crest should
alert the surgeon to the presence of
isomerism of the left atrial appendages (see
Chapter 6). As already discussed, the sinus
node almost always lies immediately
subepicardially within the terminal groove.
20
Sup.
Superior caval vein
Right
Left
Inf.
Systemic venous sinus
Right atrial
appendage
Right
pulmonary
veins
Waterstons
groove
Fig. 2.12 The heart is photographed from the right side to show
Inferior caval vein
Crest of appendage
the location of the terminal groove (black dotted line) between the
appendage and the systemic venous sinus.
Left
Sup.
Inf.
Right
Appendage
ICV
SCV
Sinus node
Terminal groove
Fig. 2.13 The cartoon shows the usual site of the sinus node within
the terminal groove (upper panel). The lower panel shows the
horseshoe arrangement found in about one-tenth of cases. SCV,
superior caval vein; ICV, inferior caval vein.
Horseshoe node
Cigar-shaped, it usually lies to the right of

the crest as seen by the surgeon; in other
words, lateral and inferior to the superior
cavoatrial junction (Figure 2.13). In about
one-tenth of cases, the node extends across

the crest into the interatrial groove. It is
then draped across the cavoatrial junction
in horseshoe fashion (Figures 2.14, 2.15)1.
Also of signicance is the course of

the artery to the sinus node (Figure 2.18).
This artery is a branch of the right coronary
artery in about 55% of individuals, and a
21
Crest of appendage
Aorta
Left
Fig. 2.14 Operative view through a median sternotomy

Sup.
Superior
caval vein
Right
demonstrating the usual site of the sinus node. The node can often
be seen as a pale cigar-shaped area located anterolaterally in the
terminal groove. This anticipated site is highlighted in the
photograph by the white cross-hatched area.
Roof of left atrium
Left
Sup.
Inf.
Inf.
Right

Sinus nodal artery
Crest of right atrial appendage
branch of the circumex artery in the

remainder2. Irrespective of its origin, it
usually courses through the anterior
interatrial groove towards the superior
cavoatrial junction (Figure 2.19),
frequently running within the atrial
myocardium. The artery usually takes its
origin from the proximal segment of its
parent coronary artery (Figure 2.20). A
signicant variant is found when the artery
a sinus node arranged in horseshoe fashion across the crest of the

right atrial appendage, with one limb in the terminal groove and
the other extending towards the interatrial groove. The nodal
location is again highlighted by the white cross-hatched area. Note
the course of the artery to the node.
originates from either coronary artery some

distance from the aorta. If taking origin
from the right coronary artery, it courses
over the lateral surface of the appendage to
reach the terminal groove (Figure 2.21). If
originating from the circumex artery, it
crosses the roof of the left atrium
(Figure 2.22). Such lateral origin is rare in
normal hearts3,4, but more frequent in
association with congenital malformations5.
Irrespective of its origin, as it enters the

sinus node, the artery may cross the crest of
the appendage, course retrocavally
(Figure 2.23), or even divide to form an
arterial circle around the junction
(Figure 2.24). All these variations should
be taken into account when planning the
safest right atrial incision, particularly
when the nodal artery crosses the lateral
margin of the right appendage, or courses
22
Superior caval vein
Waterstons groove
Inferior caval vein
Left
Sup.
Right
Pulmonary veins
Terminal crest
Inf.
Fig. 2.16 Waterstons groove seen through a median sternotomy.

The tissue plane between the atrial chambers has been partially
dissected.
Deficient oval fossa
Left
Fig. 2.17 Operative view through a median sternotomy having

Sup.
Inf.
Right
Pectinate muscles
over the roof of the left atrium. Although it

might seem obvious, care should be taken
to ensure that the incision cuts across
neither the terminal crest nor the right
coronary artery (Figure 2.25).
Opening the atrium through the most
appropriate incision shows that the
terminal groove is the external counterpart
of a prominent internal muscle bundle, the
terminal crest. This separates the pectinate
opened the right atrium. The terminal crest is seen giving rise to the
pectinate muscles of the right atrial appendage. Note that, in this
patient, the oor of the oval fossa is decient, producing an atrial
septal defect.
muscles of the appendage from the smooth

walls of the systemic venous sinus
(Figure 2.17). The cardiomyocytes are
aligned along the long axis of the crest,
which is one of the major routes for
conduction from the sinus node towards
the atrioventricular node. Anteriorly, the
crest curves in front of the orice of the
superior caval vein, with its medial
extension forming the border between the
appendage and the superior rim of the oval

fossa. The crest continues through the
superior interatrial groove as Bachmanns
bundle, the major route for conduction into
the left atrium. On rst sight, when
inspecting the right atrium through this
incision, there appears to be an extensive
septal surface between the openings of the
caval veins and the orice of the tricuspid
valve (Figure 2.26). The apparent extent of
23
Anterocaval course
Origin from right coronary artery
Retrocaval course
Arterial circle
Distal origin from right coronary artery
Fig. 2.18 The cartoon, drawn in anatomical

orientation, shows the variations in the origin of
the artery to the sinus node, and the variability
Origin from circumflex artery
relative to the cavoatrial junction. The left-hand
panels show the usual arrangement with the
origin from the right coronary artery, found in
55% of the population, with the rare variant of a
distal origin with coursing across the appendage
(lower left-hand panel). The right-hand panels
show a proximal origin from the circumex
artery, found in around 45% of the population,
with the rare variant of a distal origin with
coursing across the dome of the left atrium. The
middle panels show the variation relative to the
superior cavoatrial junction. The sinus node is
Distal origin from circumflex artery
shown in green.
Roof of left atrium
Aorta
Left
Sup.
Artery to sinus node
Inf.
Right
this septum is spurious6,7. The true

septum7,8 is conned to the oor and the
anteroinferior margin of the oval fossa
(Figures 2.27 and 2.28). The extensive
superior rim of the fossa is produced by the
folds of the interatrial groove, which

the artery to the sinus node, which in this case originates from the
circumex coronary artery and extends across the dome of the left
atrium.
separate the mouth of the superior caval

vein and the entrance of the pulmonary
veins to the left atrium (Figures 2.28). The
posteroinferior rim is another fold, this
time formed by reection of the
musculature forming the mouth of the
coronary sinus and the orice of the

inferior caval vein (Figure 2.29). These
muscular structures continue anteriorly
within the atrium as the Eustachian ridge.
This is seen to advantage when the oor of
the oval fossa is itself decient
24
Aorta
Left
Inf.
Sup.

the artery to the sinus node originating proximally from the right
coronary artery.
Right
Crest of appendage
Left
Sup.
Inf.
Right

the artery to the sinus node originating distally from the right
coronary artery and coursing over the lateral surface of the right
atrial appendage. The site of the sinus node is shown by the white
cross-hatched area.
25
Superior caval
vein
Artery to
sinus node
Dome of left atrium
Sup.
Left
Right
Retrocaval course
Fig. 2.22 In this specimen, seen in anatomical orientation, the

artery to the sinus node originates laterally from the circumex
coronary artery and courses over the dome of the left atrium. The
site of the sinus node is shown by the white cross-hatched area.
Inf.
Crest of appendage
Left
Sup.
Inf.

Right
Retrocaval nodal artery
(Figure 2.30). Because of the limited extent

of these septal components, it is an easy
matter for the surgeon to pass outside the
heart when attempting to gain access to the
left atrium through a right atrial approach.
In addition to the position of the sinus
node, and the extent of the atrial septum,
the other major area of surgical signicance
a retrocaval course of the artery to the sinus node, the site of the
node itself being emphasised by the white cross-hatched area.
within the right atrium is the site of the

atrioventricular node. This is contained
within the triangle of Koch9. This
important landmark is bounded by the
tendon of Todaro, the attachment of the
septal leaet of the tricuspid valve, and the
orice of the coronary sinus (Figure 2.31).
The tendon of Todaro9 is a brous
structure formed by the junction of the

Eustachian valve, the valve of the inferior
caval vein, and the Thebesian valve, the
valve of the coronary sinus. The brous
continuation of these two valvar structures
buries itself in the anterior continuation of
the Eustachian ridge. It then runs medially
as the tendon of Todaro before inserting
26
Retrocaval branch
Left
Sup.
Anterocaval branch
Inf.
the artery to the sinus node dividing to form an arterial circle
around the cavoatrial junction.
Right
Systemic venous sinus
Right atrial appendage
Sup.
Post.
Ant.
Fig. 2.25 The heart is shown from the right side. The terminal
Waterstons groove
Terminal groove
into the atrioventricular part of the

membranous septum (Figure 2.32). The
entire atrial component of the axis of
atrioventricular conduction tissues is
contained within the connes of the
triangle of Koch. If, in hearts with normal
segmental connections, this area is
Inf.
groove and Waterstons groove form the boundaries of the

systemic venous sinus.
scrupulously avoided during surgical

procedures, the atrioventricular
conduction tissues will not be damaged.
Should the node need to be identied more
precisely, it should be remembered that the
attachment of the tricuspid valve is some
way down the surface of the septum relative
to that of the mitral valve (Figure 2.33).

The relationship between the atrial and
ventricular muscular walls within the
triangle of Koch is complex. At rst sight,
because of the off-setting of the
attachments of the mitral and tricuspid
valves, the entire muscular area seems to
27
Sup.
Oval fossa
Post.
Ant.
Inf.
Coronary sinus
Fig. 2.26 In this specimen, viewed in the anatomical position, the

white dotted circle shows the apparently extensive septal surface
within the right atrium.
Infolded posterior rim
Sup.
Post.
Fig. 2.27 The same specimen as seen in Figure 2.26 has been
Ant.
Inf.
Infolded anterior rim
interpose between the cavities of the right

atrium and the left ventricle. Indeed, in
earliest editions of the book, we described
this area as representing an atrioventricular
transected through the oval fossa. The section shows that the rims
of the oval fossa are infoldings of the atrial walls. Note the
relationship of the anterior fold to the aortic root, the right
coronary artery, and the artery to the sinus node.
muscular septum. In the oor of the

triangle, however, the atrial musculature is
separated from the underlying ventricular
myocardium by an extension of the inferior
atrioventricular groove, with the brofatty

tissue in this area insulating the atrial
from the ventricular muscular layers10.
The extent of this insulating layer can be
28
Flap valve
Left atrium
Aortic root
Infolded superior rim

Right atrium
Post.
Fig. 2.28 This heart has been sectioned in the four-chamber

Base
Apex
Ant.
Anteroinferior rim
plane, showing that the superior rim of the oval fossa is a deep
infolding producing the interatrial groove between the systemic
venous sinus of the right atrium and the entry of the pulmonary
veins into the left atrium.
Superior infolding
Sup.
Ant.
Post.
Inf.
Left atrium
demonstrated by dissecting away the

supercial atrial musculature, at the same
time revealing the location of the artery
supplying the atrioventricular node
(Figure 2.34). The larger part of Kochs
triangle as seen by the surgeon, therefore, is
Fig. 2.29 The heart shown in Figures 2.26 and 2.27 has been
sectioned in the long axis of the venous sinus, again showing that
the superior rim of the oval fossa is an infolding of the interatrial
groove. The heart is viewed in anatomical orientation from the
back.
formed by the atrial layer of an

atrioventricular muscular sandwich, rather
than a true muscular atrioventricular
septum.
Much was written in the latter part of
the twentieth century concerning the role
of allegedly specialised pathways of

myocardium in conducting the sinus
impulse to the atrioventricular node11,12.
Molecular biologists are currently showing
that, during development, it is possible to
recognise areas of the atrial myocardium on
Septal leaflet
29
Coronary sinus
Left
Sup.
Inf.
Right
Eustachian ridge
Fig. 2.30 The heart has been opened through an atriotomy, and
the interior surface of the right atrium is shown in surgical
orientation. Note the Eustachian ridge separating the mouth of the
coronary sinus from the orice of the inferior caval vein. The oor
of the oval fossa is decient, producing an atrial septal defect.
Site of membranous septum

Hinge of tricuspid valve
Sup.
Right
Left
Inf.
Site of tendon of Todaro
Coronary sinus
Fig. 2.31 This operative view through a right atriotomy shows the
location of the triangle of Koch.
the basis of their genetic lineage.

Subsequent to birth, however, all of the
atrial myocardium, apart from the nodal
components, has achieved a working
phenotype. There is no anatomical
evidence, therefore, to support suggestions
that surgical operations should be specially

modied to avoid presumed specialised
internodal tracts13. The anatomical
paradigm of tracts of myocardium modied
for conduction in the heart is provided by
the ventricular conduction system, which is
additionally insulated from the adjacent

working ventricular myocardium14. There
are no such insulated and isolated tracts
within the atrial walls15,16. The major
muscle bundles of the atrial chambers,
nonetheless, serve as preferential pathways
30
Membranous septum
Tendon of Todaro
Hinge of
tricuspid valve
Oval fossa
Eustachian valve
Sup.
Right
Left
Inf.
Sub-Thebesian pouch
Thebesian valve
viewed in anatomical orientation, to demonstrate the boundaries

of the triangle of Koch. Note the non-uniform anisotropic
arrangement of the aggregated cardiomyocytes.
Floor of oval fossa
Sup.
Right
Fig. 2.32 The endocardium has been removed from this heart,
Mitral valvar attachment
Left
Inf.
Anteroinferior rim of oval fossa
Adipose tissue
Fig. 2.33 The heart has been sectioned in the four-chamber plane
Tricuspid valvar attachment
Ventricular septum
of conduction, with the location of these

preferential pathways dictated by the
overall geometry of the chambers
(Figure 2.35). Ideally, therefore, prominent
muscle bundles, such as the terminal crest,
the superior rim of the oval fossa, the
to show the off-setting of the hinges of the tricuspid and mitral

valves. Note the adipose tissue separating the atrial and ventricular
musculatures in the area of off-setting.
myocardium of the Eustachian ridge, and

the superior interatrial fold, should be
preserved during atrial surgery. Even if
they cannot be preserved, the surgeon can
rest assured that internodal conduction will
continue as long as some strand of viable
atrial myocardium interposes between the

nodes, providing that the arterial supply to
the nodes, or the nodes themselves, are not
traumatised. The key to avoiding
postoperative atrial arrhythmias, therefore,
is the fastidious preservation of the sinus
31
Artery to AV node
Membranous septum
Right atrium
Left atrium
Fig. 2.34 This dissection, in anatomical orientation having

removed the non-coronary sinus of the aortic valve, shows the
brofatty tissue (star) interposed between the atrial and
ventricular muscular layers (yellow and blue dashed lines) of the
atrioventricular muscular sandwich. Note the artery to the
atrioventricular (AV) node.
Circumflex coronary artery
Oval fossa
Mouth of superior caval vein

Tricuspid valve
Sup.
Ant.
Post.
Inferior caval vein
Coronary sinus
and atrioventricular nodes and their

arteries, rather than concern about nonexistent tracts of purportedly specialised
atrial myocardium.
Inf.
Much is also written about the brous

skeleton of the heart. The strongest part of
this skeleton is the central brous body.
This area of brous tissue touches on three
Fig. 2.35 The right atrium, seen in anatomical

orientation, has been opened through a
window in the appendage. The muscular walls
surround several orices, producing
preferential routes of conduction (arrows).
of the four cardiac chambers, but is seen

most clearly by the surgeon when working
from the right atrium (Figure 2.36). Rather
than being considered as a specic body, it
32
Atrioventricular membranous septum

Septal leaflet
Left
Sup.
Inf.
tendon of Todaro inserting into the atrioventricular component of

the membranous septum, which forms the right margin of the
central brous body.
Right
Tendon of Todaro
Fig. 2.36 Operative view through a right atriotomy showing the
Post.
Right
Left
Ant.
Left ventricular
outflow tract
Oval fossa
Fig. 2.37 The heart is sectioned to replicate the

echocardiographic four-chamber plane. The section passes through
the membranous part of the septum, which is divided by the hinge
of the septal leaet of the tricuspid valve into atrioventricular (red
double-headed arrow) and interventricular (blue double-headed
arrow) components.
Hinge of septal leaflet
is better conceptualised as an area within

the heart where the membranous septum,
the hinges of the leaets of the
atrioventricular valves, and the brous
components of the aortic root join in

brous continuity (Figure 2.37). Its extent
is best seen from the left side, albeit that
this aspect is rarely, if ever, seen by the
surgeon (Figure 2.38). The anatomical

view shows how the rightward margin of
the area of continuity between the leaets
of the aortic and mitral valves, also known
Membranous septum
33
Aortic-mitral continuity
Sup.
Fig. 2.38 This specimen, viewed in anatomical orientation, is
Post.
opened to show the origin of the aorta from the left ventricle, with
the membranous septum in brous continuity with the leaets of
the aortic and mitral valves. Note the site of the left bundle branch,
marked by the white dots.
Ant.
Inf.
Interleaflet triangle
Aortic-mitral continuity
Membranous septum
Right fibrous trigone
as the right brous trigone, joins with the

membranous septum to form the basis of
the brous body (Figure 2.39). This view
also emphasises the intimate relationship of
the brous body to many other important
structures within the heart, particularly the
Fig. 2.39 This close-up, in anatomical orientation, shows the

relationships between the membranous septum and the aortic
valve having removed the leaets of the valve itself (black dashed
lines). The membranous septum is in continuity superiorly with the
interleaet triangle between the right and non-coronary leaets of
the aortic valve. It is also continuous with the thickened right end of
the brous continuity between the leaets of the aortic and mitral
valves, known as the right brous trigone.
brous triangle between the hinges of the

right and non-coronary leaets of the aortic
valve. The central brous body, therefore,
serves as an anatomical focal point for the
cardiac surgeon. Operations involving
valvar replacement or repair, closure of
septal defects, and control of arrhythmias

require the surgeon to understand its
anatomical relationships implicitly, from
both the obvious right (Figure 2.36), and
also the usually invisible left (Figure 2.38)
sides. Knowledge of the location of the
34
Anterosuperior leaflet
Inferior leaflet
Left
Sup.
Inf.
Fig. 2.40 This operative view through a right atriotomy, seen in

Right
Septal leaflet
atrioventricular component of the

membranous septum is also the key to
understanding the occurrence of the socalled Gerbode septal defect17, which
produces a direct shunt from the left
ventricular outow tract to the right atrium
(see Figure 2.37).
The nal atrial component, the vestibule
to the right ventricle, surrounds the orice
of the tricuspid valve, and is contiguous with
both the systemic venous component and
the appendage of the right atrium. The
anterior junction of these two parts of the
atrium overlies the peripheral attachment of
the anteroseptal commissure of the tricuspid
valve and the supraventricular crest of the
right ventricle (Figure 2.40). The posterior
junction is at the orice of the coronary
sinus, where there is usually an extensive
inferior trabeculated diverticulum.
Although usually called the post-Eustachian
sinus, it is sub-Thebesian when the heart is
viewed relative to the anatomical position
(Figure 2.35).
MORPHOLOGICALLY LEFT
ATRIUM
As with the right atrium, the left atrium
possesses an appendage, an extensive
venous component, and a vestibule. Unlike
the situation with the right atrium, it is also
surgical orientation, shows the vestibule of the right atrium and the
three leaets of the tricuspid valve.
possible to recognise the extensive body of

the left atrium (Figure 2.41). The chamber,
of course, is separated from its neighbour
by the septum. The body of the atrium is
conuent with the venous component.
Unlike in the right atrium, the narrow
junction between these parts and the
appendage is unmarked by either a terminal
groove or crest (Figure 2.41). Because of
the posterior position of the left atrium, and
its rm anchorage by the four pulmonary
veins, it can be difcult for the surgeon to
gain direct access to the left atrium, so
knowledge of the salient anatomy can help
best exposure of the cavity. Probably the
most popular route is provided by an
incision made just posterior to the right
lateral aspect of the interatrial groove
(Figure 2.42). This groove, the extensive
infolding between the right pulmonary
veins and the venous sinus of the right
atrium, produces the superior rim of the
oval fossa (Figure 2.28). A posteriorly
directed incision within this groove takes
the surgeon directly into the left atrium. If
necessary, the incision can be extended to
the superior aspect of the left atrium by
incising the pericardial fold between the
superior caval vein and the right
pulmonary artery (Figure 2.4). Because the
infolding of the interatrial groove also
forms the superior border of the oval fossa,
much the same access can be gained by

approaching through the right atrium, and
incising just superiorly within the fossa. It
must be remembered that an extensive
incision may take the surgeon out of the
connes of the atrial chambers and into the
pericardial space. Perhaps more
importantly, it should be noted that such an
incision may damage the artery to the sinus
node, either in the interatrial groove or on
the roof of the left atrium. The left atrium
can also be entered superiorly by incising
directly through its roof. If the aorta is
pulled anteriorly and to the left, an extensive
trough is seen between the two atrial
appendages (Figure 2.43), with an incision
through the oor of this trough entering the
left atrium directly. When making such an
incision, it must again be remembered that
the artery to the sinus node may be coursing
through this area when it takes origin from
the circumex artery (Figure 2.43). In other
instances, this artery may pass through the
infolding of the interatrial groove to reach
the terminal groove.
Once access is gained to the left atrium,
the small size of the opening of the
appendage is apparent (Figure 2.44). The
mouth of the appendage lies to the left of
the mitral orice as viewed by the surgeon.
Anatomically, it is positioned above the
superior end of the zone of apposition
Appendage
35
Venous component
Body
Ant.
Sup.
Inf.
Vestibule
Septum
Post.
Fig. 2.41 In this heart, shown in anatomical orientation, the left

atrium is opened to show its components. Note the extensive body.
Left
Interatrial groove
Inf.
Sup.
Right
Fig. 2.42 This operative view through a median sternotomy

Incision to left atrium
shows how an incision through the bottom of Waterstons groove

(white dotted line) takes the surgeon into the left atrium.
between the valvar leaets. The greater

part of the pulmonary venous sinus,
conuent with the smooth-walled atrial
body, will usually be located inferiorly,
away from the operative eld. It is the
vestibule of the mitral orice that
dominates the picture (Figure 2.45). The
septal aspect will be anterior, exhibiting the

typically roughened apvalve aspect of its
left side (Figure 2.46). The large sweep of
tissue between the ap valve of the septum
and the opening of the appendage is the
internal aspect of the deep anterior
interatrial groove.
MORPHOLOGICALLY RIGHT
VENTRICLE
The musculature of the right ventricle
extends from the atrioventricular to the
ventriculoarterial junctions.
Understanding of ventricular morphology
36
Dome of left atrium
Aorta
Right atrial
appendage
Left
Sup.
Inf.
Right
shows how retraction of the aorta to the left reveals the deep
trough inferior to the right pulmonary artery and between the
atrial appendages. Note that, in this heart, the artery to the sinus
node courses through this trough.
Right pulmonary
artery
Mouth of appendage
Pulmonary
venous component
Body of atrium
Sup.
Fig. 2.44 The left atrium is photographed from behind to show

Right
Left
Vestibular myocardium
Fenestrated
oval fossa
in general is greatly aided by considering

the ventricles in terms of three
components17. These are the inlet, apical
trabecular, and outlet parts, respectively
Inf.
the relations of the mouth of the left atrial appendage. All

components of the atrial chamber can be seen. Note the isthmus
formed by the vestibular myocardium, and the extensive fold
between the mouth of the appendage and the origin of the left
pulmonary veins from the pulmonary venous component (star).
(Figure 2.47). The inlet portion of the right

ventricle contains, and is limited by, the
tricuspid valve and its tension apparatus.
Although not always easy, it is generally
possible to distinguish three leaets in the

valvar orice. They lie in anterosuperior,
septal, and inferior or mural locations
(Figures 2.48, 2.49). When seen in the
37
Ant.
Aortic leaflet
Sup.
Inf.
Post.
Mural leaflet
Vestibule
Fig. 2.45 On gaining access through a right-sided left atriotomy,

the vestibule of the mitral orice dominates the surgical picture.
Note the location of the leaets of the mitral valve.
Body of atrium
Atrial septum
Sup.
Post.
Ant.
Inf.
Mitral valvar vestibule
closed position, their boundaries are clearly

marked by their zones of apposition, which
extend to the centre of the valvar orice.
The entirety of the zones of apposition
could, logically, be dened as the
commissures. It is the peripheral parts of
the zones of apposition that are usually
described as the commissures18, albeit that
in the atrioventricular valves these areas do
Fig. 2.46 The left atrium is windowed from the left and posterior
aspect, showing its component parts. Note the probe in the patent
foramen.
not abut directly on the valvar annulus.

The zones of apposition between the
leaets in these commissural areas are
usually tethered by fan-shaped cords
arising atop prominent papillary muscles.
We discuss the details of this valvar and
commissural anatomy in our next chapter.
In anatomical terms, the most constant
distinguishing morphological feature of the
tricuspid valve is the direct attachments to

the septum of the cords tethering its septal
leaet (Figure 2.49).
The trabecular component of the right
ventricle extends to the apex, where its wall
is particularly thin, being especially
vulnerable to perforation by cardiac
catheters and pacemaker electrodes. When
compared to those of the left ventricle, the
38
Supraventricular crest
Septomarginal
trabeculation
Outlet
Inlet
Apical
trabecular
component
Base
Right
Left
Fig. 2.47 This specimen, viewed in anatomical orientation, shows
Apex
the three components of the morphologically right ventricle.
Medial papillary muscle
Sup.
Left
Right
Inf.
Anterosuperior
leaflet
Septal leaflet
Tricuspid
valve
Fig. 2.48 The right atrioventricular junction is photographed

Inferior leaflet
from the ventricular apex, showing the location of the leaets of

the tricuspid valve.
right ventricular apical trabeculations are

uniformly coarse. The outlet component of
the right ventricle is a complete muscular
sleeve, the infundibulum, which extends
from the ventricular base to support the
leaets of the pulmonary valve. The leaets
of the valve are attached to the infundibular

musculature in semilunar fashion. These
hinges cross the circular anatomical
ventriculoarterial junction so as to
incorporate triangles of arterial wall within
the ventricle, and crescents of ventricular
muscle in the bases of the pulmonary valvar

sinuses (Figure 2.50). Another
distinguishing morphological feature of
the right ventricle is the prominent
muscular shelf that interposes between the
tricuspid and pulmonary valvar orices.
39
Left
Sup.
Inf.
Right
Septal leaflet
Fig. 2.49 Operative view through a right atriotomy showing the
Direct septal attachments
Anatomical ventriculoarterial junction
direct cordal attachments to the septum of the septal leaet of the

tricuspid valve.
Sup.
Right
Left
Inf.
Fig. 2.50 In this heart, shown in anatomical orientation, the

leaets of the pulmonary valve have been removed. Note how the
semilunar attachment of the pulmonary valvar leaets crosses the
anatomical ventriculoarterial junction, the dotted lines and crosshatchings emphasising the crescents of ventricular muscle
incorporated in the valvar sinus and the triangles of arterial wall
within the ventricle.
Haemodynamic ventriculoarterial junction
This is the supraventricular crest. At rst

sight (Figure 2.51), it has the appearance
of a thick muscle bundle, but its larger part
is no more than the infolded inner heart
curve (Figure 2.52). Incisions, or deep
sutures, through this part run into the
transverse sinus and right atrioventricular
groove, and can jeopardise the right
coronary artery19. Only a small portion of
the most medial part of the crest can be

removed so as to create a hole between the
subpulmonary and subaortic outow
tracts (Figure 2.52). This part is a true
muscular outlet septum, but cannot be
distinguished from the remainder of the
crest by gross inspection (Figure 2.51).
The crest continues distally as the freestanding infundular muscular sleeve
(Figure 2.53). It is the presence of this

sleeve that permits the valve itself to be
removed as an autograft for use in the Ross
procedure (Figures 2.54, 2.55).
Considered as a whole, the
supraventricular crest inserts between the
limbs of an equally prominent and
important right ventricular septal
trabeculation. This structure, described
40
Septoparietal
trabeculations
Sup.
Left
Fig. 2.51 In this anatomical specimen, viewed in surgical
Right
Inf.
orientation, the prominent muscular supraventricular crest is seen

between the leaets of the tricuspid and pulmonary valves. Note
the medial papillary muscle arising from the septomarginal
trabeculation.
Septomarginal trabeculation
Sup.
Septomarginal
trabeculation
Left
Right
Inf.
Pulmonary valve
Right
coronary
artery
Fig. 2.52 Dissection of the specimen shown in Figure 2.43 shows
Resected outlet septum
Ventriculoinfundibular fold
by us as the septomarginal trabeculation,

but also known as the septal band, has
anterosuperior and posteroinferior limbs
that clasp the septal attachment of the
crest (Figure 2.56). The anterosuperior
here that most of the supraventricular crest is the inner curvature of

the heart, or the ventriculoinfundibular fold. Note the location of
the right coronary artery. The site occupied by the outlet septum
has been resected.
limb runs up to the attachment of the

leaets of the pulmonary valve, while the
posteroinferior limb extends backwards,
extending inferior to the interventricular
component of the membranous septum,
and reinforcing the muscular septum. The

medial papillary muscle usually arises
from this posteroinferior limb. The body
of the septomarginal trabeculation itself
extends towards the apex of the ventricle,
41
Free-standing infundibulum
Pulmonary valve
Aortic root
Sup.
Left
Right
Inf.
Septomarginal
trabeculation
Fig. 2.53 Further dissection of the heart shown in Figures 2.51

and 2.52 reveals the free-standing sleeve of infundibular muscle
supporting the leaets of the pulmonary valve.
Anterior interventricular artery
First septal perforator

Aortic valve
Left
Ant.
Post.
Fig. 2.54 In this heart, the pulmonary trunk
Right
breaking up into a sheath of smaller

trabeculations. Some of these mingle into
the apical trabecular portion, and some
support the tension apparatus of the
tricuspid valve. Several of these
trabeculations are often particularly
Infundibular sleeve
has been turned forwards to show the sleeve of

free-standing infundibular musculature.
prominent. One becomes the anterior

papillary muscle, while another extends
from the septomarginal trabeculation to
the anterior papillary muscle, being
termed the moderator band. Additional
signicant right ventricular trabeculations
arise from the anterior margin of the

septomarginal trabeculation. Variable in
number, these are the septoparietal
trabeculations (Figure 2.56).
It is the uniformly coarse apical
trabeculations that, for the morphologist,
42
Left coronary artery
Aortic valve
Left
Sup.
Inf.
Right ventricular
infundibular sleeve
Right
Fig. 2.55 In the heart shown in Figure 2.54, again viewed from
above and from the right, the pulmonary infundibulum has been
removed from the base of the heart, revealing the free-standing
myocardial sleeve that lifts the valvar leaets away from the left
ventricle. Note the site of the rst septal perforating artery.
Septomarginal
trabeculation
Sup.
Left
Right
Fig. 2.56 The right ventricle has been opened from the front,
Inf.
Anterior papillary muscle
with the heart orientated anatomically, to show the septoparietal

trabeculations (stars). The most inferior of the trabeculations is the
moderator band.
43
Ant.
Left
Post.
Coarse right
ventricular
trabeculations
Right
Fig. 2.57 The apical component of the ventricular mass has been
Fine left ventricular trabeculations
transected, and is photographed from above to show the different

patterns of the apical trabeculations of the two ventricles.
Sup.
Post.
Ant.
Inf.
Outlet
Inlet
Fig. 2.58 In this specimen, viewed from the side in anatomical

orientation, the morphologically left ventricle is opened in
clam-shell-like fashion to show its three components.
Apical trabecular component
serve as the most constant anatomical

feature of the morphologically right
ventricle. Taken overall, there are a
number of morphological differences
between the ventricles that permit their
distinction. These include the arrangement
of the leaets of the atrioventricular valves
and their tension apparatus, the ventricular
shape, the thickness of the ventricular
walls, and the conguration of the outow
tracts. These features, however, can be

altered or lacking in the congenitally
abnormal heart. In nal arbitration of
ventricular morphology, therefore, the
morphologist relies on the contrast, in the
same heart, between the coarse
trabeculations of the right ventricle, and
the much ner trabeculations seen in the
apical part of the left ventricle
(Figure 2.57).
MORPHOLOGICALLY LEFT
VENTRICLE
As with the right ventricle, the musculature
of the left ventricle extends from the
atrioventricular to the ventriculoarterial
junctions. It is again conveniently
considered in terms of inlet, apical
trabecular, and outlet components
(Figure 2.58). In the left ventricle, there is
44
Base
Right
Left
Apex
Aortic leaflet
Mural leaflet
Fig. 2.59 This specimen, viewed from behind, and opened

Tendinous cords
Papillary muscles
Sup.
through a cut in the left atrioventricular junction adjacent to the

septum, demonstrates the marked differences in the arrangement
of the aortic and mural leaets of the mitral valve.
Posterosuperior muscle
Base
Apex
Inf.
Fig. 2.60 This specimen, viewed from behind after the parietal
Anteroinferior muscle
marked overlapping of the inlet and outlet

components. The inlet component
surrounds, and is limited by, the mitral
valve and its tension apparatus. Its two
leaets, supported by two prominent
papillary muscles and their tendinous
cords, have widely differing appearances
(Figure 2.59). The anterosuperior leaet is
short, squat, and relatively square. This
leaet, in brous continuity with the aortic
valve, is best termed the aortic leaet. The
other leaet is narrower, and its junctional
attachment more extensive, being
supported by the parietal part of the left
wall of the left ventricle has been removed, is dissected to show the
adjacency of the anteroinferior and posterosuperior papillary
muscles of the mitral valve.
atrioventricular junction. Positioned

posteroinferiorly, it is accurately termed
the mural leaet. Because the aortic leaet
of the mitral valve forms part of the outlet
of the left ventricle, the boundary between
the inlet and outlet is somewhat blurred.
The papillary muscles of the valve, located
in anteroinferior and posterosuperior
positions, are close to each other at their
origin (Figure 2.60). It is incorrect to
describe the muscles as being anteroseptal
and posterolateral, these terms reecting
the bad habit of morphologists of removing
the heart from the body and describing it as
positioned on its apex20. Unlike the

tricuspid valve, the leaets of the mitral
valve have no direct septal attachments,
because the deep posteroinferior
diverticulum of the subaortic outow tract
displaces the aortic leaet of the valve away
from the muscular ventricular septum
(Figure 2.61). The trabecular component
of the left ventricle extends to the
ventricular apex and has characteristically
ne trabeculations (Figure 2.57). As in the
right ventricle, the myocardium is
surprisingly thin at the apex. This feature is
important to the cardiac surgeon, who may
Sup.
Post.
45
Aorta
Ant.
Inf.
Mitral valve
Right atrium
Inferior extension
have reason to place catheters and

electrodes in the right ventricle, or drainage
tubes in the left side. Immediate
perforation, or delayed rupture, may occur.
This may be a particular problem should
catheters stiffened by hypothermia be
pushed against the apical endocardium as
the heart is manipulated during surgery on
the coronary arteries21.
The outlet component of the left
ventricle supports the aortic valve. Unlike
its right ventricular counterpart, it is not a
complete muscular structure. The septal
wall is composed largely of muscle, but in
this area the membranous septum is found,
forming part of the central brous body in
the subaortic outow tract (Figure 2.39).
The posterolateral portion of the outow
tract is composed exclusively of brous
tissue, namely the brous curtain joining
the leaets of the aortic valve to the aortic
leaet of the mitral valve. The left lateral
quadrant, continuing around to the
septum, is a muscular structure
representing the lateral margin of the inner
heart curvature, and separating the cavity
of the left ventricle from the transverse
sinus. The muscular septal surface of the
outow tract is characteristically smooth,
with the fan-like left bundle branch
cascading down from the septal crest. The
Fig. 2.61 This heart, seen from above and from the right in
anatomical orientation, has been prepared by removing the atrial
musculature and the non-coronary sinus of the aortic valve to
illustrate the inferior extension of the subaortic outow tract
separating the mitral valve from the septum.
landmark of its descent is the membranous

septum immediately beneath the zone of
apposition between right coronary and
non-coronary leaets of the aortic valve
(Figure 2.62). The bundle descends,
initially, as a relatively narrow solitary
fascicle, but soon divides into three
interconnected fascicles that radiate into
anterior, septal, and posterior divisions.
The interconnecting radiations do not fan
out to any degree until the bundle itself has
descended to between one-third and onehalf the length of the septum. As with the
pulmonary valve, the leaets of the aortic
valve are not attached in circular fashion to
a supporting ring of brocollagenous
tissue. Instead, the leaets are attached in
semilunar fashion, with the hinge lines
crossing the anatomical ventriculoaortic
junction (Figure 2.63). This arrangement
again means that crescents of ventricular
muscle are incorporated into the bases of
two of the aortic sinuses, while three
triangles of brous tissue are created
beneath the sinutubular junction22.
THE AORTA
The ascending aorta begins at the distal
extremity of the three aortic sinuses, the
sinutubular junction, which lies at the line
of opening of the free edge of the leaets of

the aortic valve (Figure 2.64). The
ascending aortic trunk runs its short
course, passing superiorly, and obliquely to
the right, and slightly forwards, towards
the sternum. It is contained within the
brous pericardial sac, so its surface is
covered with serous pericardium. Its
anterior surface abuts directly on the
pulmonary trunk, which is also covered
with serous pericardium. The two vessels
together make up the vascular pedicle of
the heart (Figure 2.65). The ascending
aorta is related anteromedially to the right
atrial appendage, and posterolaterally to the
right ventricular outow tract and the
pulmonary trunk. Extrapericardially, the
thymus gland lies between it and the
sternum. The medial wall of the right
atrium, the superior caval vein, and the
right pleura relate to its right side. On the
left, its principal relationship is with the
pulmonary trunk. Posterior to the
ascending aorta lies the transverse sinus of
the pericardium (Figure 2.3), which
separates it from the roof of the left atrium
and the right pulmonary artery. The arch
of the aorta begins at the superior
attachment of the pericardial reection
just proximal to the origin of the
brachiocephalic artery. It continues
46
Right coronary leaflet
Sup.
Non-coronary leaflet
Right
Left
Inf.
Mitral valve
Fig. 2.62 This specimen, orientated in the anatomical position,
Membranous septum
demonstrates the relationship of the zone of apposition between

the right and non-coronary leaets to the membranous septum
and the interleaet triangle between the right coronary and noncoronary leaets of the aortic valve.
Anatomic ventriculoarterial junction
Ant.
Base
Fig. 2.63 This dissection demonstrates that, as in the pulmonary
Apex
Post.
Haemodynamic ventriculoarterial junction
superiorly for a short distance, before

coursing posteriorly and to the left,
crossing the lateral aspect of the distal
trachea, and nally terminating on the
lateral aspect of the vertebral column.
Here, it is tethered by the parietal pleura
and the arterial ligament. During its
course, it gives off the brachiocephalic,
the left common carotid, and the left
subclavian arteries (Figure 2.66).
Bronchial arteries arise from the
underside of the arch (Figure 2.67).
They can be particularly troublesome
valve (see Figure 2.50), the attachments of the leaets of the aortic
valve (black dotted line) cross the anatomical ventriculoarterial
junction (black dashed line), incorporating crescents of ventricular
tissue into the arterial valvar sinuses, and triangles of arterial tissue
into the ventricular base.
if not carefully identied in the presence

of aortic coarctation. The left phrenic and
vagus nerves run over the anterolateral
aspect of the arch just beneath the
mediastinal pleura. The left recurrent
laryngeal nerve takes origin from the
vagus. It curls superiorly around the
arterial ligament before passing on to the
posteromedial side of the arch. Here, the
arch relates to the tracheal bifurcation and
oesophagus on its medial border, but also
to the left main bronchus and the left
pulmonary artery inferiorly.
The descending or thoracic aorta

continues from the arch, running an initial
course lateral to the vertebral bodies, and
reaching an anterior position at its
termination. It gives off many branches to
the organs of the thorax throughout its
course, as well as the prominent lower nine
pairs of intercostal arteries. These latter
vessels are of critical concern for the cardiac
surgeon (see Chapter 9). In coarctation of
the aorta, they serve as primary collateral
vessels to bypass the obstructed aorta,
accounting for the rib notching seen in
Sinutubular junction
Ascending aorta
Aortic sinus
Transverse sinus
Left atrium
Left ventricle
Ant.
Base
Apex
Post.
Fig. 2.64 This long axis section shows the

sinutubular junction at the line of peripheral
attachment of the zones of apposition
between the leaets of the aortic valve.
Left
Detached pericardial reflection
Inf.
Sup.
Right
Pulmonary trunk
Ascending aorta
older children with this lesion. These

vessels, and their branches to the chest
wall, can be a source of troublesome
bleeding if not properly secured when
operating on such patients. The surgeon
must also remember that the dorsal
branches of the intercostal vessels
contribute a spinal branch that is important
in supplying blood to the spinal cord.
shows the vascular pedicle leading to the beginning of the aortic

arch, which gives rise to the brachiocephalic and left common
carotid arteries just distal to the detached pericardial reection
(white dotted line). The origin of the left subclavian artery is
not seen.
Because it is difcult to predict exactly the

site of origin of these vital branches, the
surgeon must make every attempt to
protect their origin from permanent
occlusion. The important bronchial arteries
(Figure 2.67) also arise from the
descending segment of the thoracic
aorta. These vessels can become dilated in
the presence of pulmonary atresia, when
they serve as a source of pulmonary

vascular supply.
THE PULMONARY ARTERIES

The pulmonary trunk is a short vessel,
usually less than ve centimetres in length
in the adult (Figure 2.68). It is completely
contained within the pericardium
47
48
Brachiocephalic arteries
Ant.
Left
Right
Post.
Right pulmonary artery
Pulmonary trunk
Aorta
Left pulmonary artery
Fig. 2.66 The arterial trunks have been removed from the base of
the heart, and photographed from the apical aspect, showing their
patterns of branching.
Arterial ligament
Bronchial artery
Aortic arch
Trachea
Left
Inf.
Sup.
Right pulm. artery

Right
(Figure 2.69) and, similar to its running

mate, the ascending aorta, it is covered with
a layer of serous pericardium except where
the two vessels abut each other in the
Fig. 2.67 This dissection, viewed from the right side in surgical
orientation, shows a bronchial artery arising from the aorta in the
midline and dividing to supply both bronchuses. Pulm., pulmonary.
vascular pedicle. It takes origin from the

most anterior aspect of the heart, lying just
behind the lateral edge of the sternum and
the second left intercostal space. Initially,
the pulmonary trunk overlies the aorta and

left coronary artery, but it soon moves to a
side-by-side relationship with the
ascending aorta. The left coronary artery
Pulmonary trunk
49
Infundibulum
Left
Inf.
Sup.
Fig. 2.68 This operative view, taken through a median
Right
Pulmonary trunk
sternotomy, shows the short course of the pulmonary trunk prior to

its bifurcation.
Pericardial reflection
Ligament of Marshall
Sup.
Ant.
Post.
Fig. 2.69 The anatomical specimen is shown from the left side,
Infundibulum
Inf.
turns abruptly anteriorly to lie between the

left atrial appendage and the pulmonary
trunk. The arterial ligament extends from
the aorta to the very end of the pulmonary
trunk as the latter divides into left and right
pulmonary arteries. The left pulmonary
artery then courses laterally in front of the
illustrating the short intrapericardial course of the pulmonary

trunk. Note the location of the ligament of Marshall.
descending aorta and the left main-stem

bronchus before it sends branches to the
hilum of the lung. Posteroinferiorly, the
left pulmonary artery is connected to the
left superior pulmonary vein by a fold of
serous pericardium that contains a
ligamentous remnant of the left superior
caval vein, the ligament of Marshall

(Figure 2.69). The right pulmonary artery
is somewhat longer than the left, having to
traverse the mediastinum beneath the
aortic arch, and then behind the superior
caval vein, to reach the hilum of the lung
(Figure 2.70). It lies in a posteroinferior
50
Right pulmonary
artery
Superior
caval vein
Left pulmonary
artery
Aorta
Post.
Right
Left
Fig. 2.70 The heart has been sectioned in the short axis and
Ant.
Pulmonary trunk
Pulmonary valve
position relative to the azygos vein, and is

anterior to the left main bronchus. The
right pulmonary artery often branches
before reaching the lateral wall of the
superior caval vein posterior to the
transverse sinus of the pericardium. In
this situation, a large upper lobar branch
may be mistaken for the right pulmonary
artery itself.
References
1. Anderson KR, Ho SY, Anderson RH. The
location and vascular supply of the sinus
node in the human heart. Br Heart J 1979;
41: 2832.
2. James TN. Anatomy of the Coronary
Arteries. New York, NY: Hoeber, 1961;
pp 103106.
3. McAlpine WA. Heart and Coronary Arteries.
An Anatomical Atlas for Clinical Diagnosis,
Radiological Investigation and Surgical
Treatment. New York, NY: Springer
Verlag, 1975; p 152.
4. Busquet J, Fontan F, Anderson RH, Ho SY,
Davies MJ. The surgical signicance of the
atrial branches of the coronary arteries. Int
J Cardiol 1984; 6: 223234.
5. Barra Rossi M, Ho SY, Anderson RH, Rossi
Filho RI, Lincoln C. Coronary arteries in
complete transposition: the signicance of
the sinus node artery. Ann Thorac Surg 1986;
42: 573577.
photographed from above, showing the branching pattern of the

pulmonary trunk.
6. Sweeney LJ, Rosenquist GC. The normal

anatomy of the atrial septum in the human
heart. Am Heart J 1979; 98: 194199.
7. Anderson RH, Webb S, Brown NA.
Clinical anatomy of the atrial septum with
reference to its developmental components.
Clin Anat 1999; 12: 362374.
8. Anderson RH, Brown NA. The anatomy of
the heart revisited. Anat Rec 1996; 246: 17.
9. Ho SY, Anderson RH. How constant is the
tendon of Todaro as a marker for the
triangle of Koch? J Cardiovasc
Electrophysiol 2000; 1: 8389.
10. Anderson RH, Ho SY, Becker AE. Anatomy
of the human atrioventricular junctions
revisited. Anat Rec 2000; 260: 8191.
11. James TN. The connecting pathways
between the sinus node and the AV node
and between the right and the left atrium
in the human heart. Am Heart J 1963; 66:
498508.
12. James TN, Sherf L. Specialized tissues and
preferential conduction in the atria of the
heart. Am J Cardiol 1971; 28: 414427.
13. Isaacson R, Titus JL, Merideth J, Feldt
RH, McGoon DC. Apparent interruption
of atrial conduction pathways after
surgical repair of transposition of the
great arteries. Am J Cardiol 1972; 30:
533535.
14. Anderson RH, Ho SY. Anatomic criteria
for identifying the components of the axis
responsible for atrioventricular conduction.
15.
16.
17.
18.
19.
20.
21.
22.
J Cardiovasc Electrophysiol 2001; 12:

12651268.
Janse MJ, Anderson RH. Internodal atrial
specialised pathways fact or ction? Eur
J Cardiol 1974; 2: 117137.
Anderson RH, Ho SY, Smith A, Becker
AE. The internodal atrial myocardium.
Anat Rec 1981; 201: 7582.
Gerbode F, Hultgren H, Melrose D,
Osborn J. Syndrome of left ventricular
right atrial shunt. Ann Surg 1958; 148:
433446.
Frater RWM, Anderson RH. How can we
logically describe the components of the
arterial valves? J Heart Valve Dis 2010; 19:
438440.
McFadden PM, Culpepper WS,
Ochsner JL. Iatrogenic right ventricular
failure in tetralogy of Fallot repairs:
reappraisal of a distressing problem. Ann
Thorac Surg 1982; 33: 400402.
Anderson RH, Frater RWM. Editorial.
How can we best describe the components
of the mitral valve? J Heart Valve Dis 2006;
15: 736739.
Breyer RH, Lavender S, Cordell AR.
Delayed left ventricular rupture secondary
to transatrial left ventricular vent. Ann
Thorac Surg 1982; 3: 189191.
Sutton JP 3rd, Ho SY, Anderson RH.
The forgotten interleaet triangles: a
review of the surgical anatomy of the
aortic valve. Ann Thorac Surg 1995; 59:
419427.
Surgical anatomy
of the valves of
the heart
52
It is axiomatic that a thorough knowledge of

valvar anatomy is a prerequisite for
successful surgery, be it valvar replacement
or reconstruction. The surgeon will also
require a rm understanding of the
arrangement of other aspects of cardiac
anatomy to ensure safe access to a diseased
valve or valves. These features were
described in the previous chapter.
Knowledge of the surgical anatomy of the
valves themselves, however, must be
founded on appreciation of their
component parts, the relationships of the
individual valves to each other, and their
relationships to the chambers and arterial
trunks within which they reside. This
requires understanding of, rstly, the basic
orientation of the cardiac valves,
emphasising the intrinsic features that
make each valve distinct from the others.
This information must be supplemented by
attention to their relationships with other
structures that the surgeon must avoid,
notably the conduction tissues and the
major channels of the coronary circulation.
For this chapter, throughout our narrative
we will presume the presence of a normally
structured heart, lying in its usual position,
and without any coexisting congenital
cardiac malformations.
THE VALVAR COMPLEXES

When considering the valves, we
distinguish between the atrioventricular
valves, which guard the atrioventricular
junctions, and the arterial valves, which
guard the ventriculoarterial junctions
(Figure 3.1). The atrioventricular valves
are best analysed in terms of the valvar
complex, made up of the annulus, the
leaets, the tendinous cords, the papillary
muscles, and the supporting ventricular
musculature (Figure 3.2). All of these
components must work in harmony so as
to achieve valvar competence1. The
leaets of the atrioventricular valves are
supplied with a complex tension
apparatus, as they must withstand the full
force of ventricular systole, so as to retain
their competence when in their closed
position. The arterial valves are also a
combination of complex anatomical parts.
Often named the semilunar valves, it is
the leaets that are semilunar, being
hinged within the overall valvar complex,
which extends from the proximal
ventriculoarterial orice, a virtual ring
constructed at the nadir of the hinges of
the leaets, to the sinutubular junction
(Figure 3.3). The components of the
valvar complex are the leaets, their

supporting arterial sinuses, and the
brous interleaet triangles. In the right
ventricle, the arterial sinuses are
supported at the ventricular base
exclusively by infundibular myocardium.
The junction between the myocardial
walls and the broelastic walls of the
sinuses, the anatomical ventriculoarterial
junction, forms one of the two true
anatomical rings to be found within the
pulmonary root (Figure 3.4). The other
obvious anatomical ring is the sinutubular
junction. In the left ventricle, only the two
coronary arterial valvar sinuses are
supported by ventricular musculature,
with the sinus and leaet of the noncoronary aortic sinus being contiguous
with one of the leaets of the mitral valve
(Figure 3.5). It is still possible,
nonetheless, to recognise the ring formed
at the junction between the ventricular
supporting structures and the broelastic
walls of the sinuses, and to recognise the
brous interleaet triangles extending
distally to the level of the sinutubular
junction. As in the pulmonary root, the
sinutubular junction is the other obvious
anatomical ring to be found within the
aortic root (Figures 3.4, 3.5). In both of
Sup.
Arterial valve
Apex
Base
Inf.
Left ventricle
Aorta
Left atrium
Fig. 3.1 The heart has been sectioned in the
Atrioventricular valve
long axis plane, and orientated so as to

replicate the long axis parasternal section as
obtained by echocardiographers. The cut
clearly shows how, in the left heart, the mitral
valve guards the atrioventricular junction, and
the aortic valve the ventriculoarterial junction.
Surgical anatomy: valves of the heart
53
Sup.
Apex
Base
Valvar
leaflets
Inf.
Tendinous
cords
Papillary
muscles
Attachments
at annulus
Ventricular musculature
Valvar
sinuses
taken from the long axis section illustrated in

Figure 3.1, shows the components of the
atrioventricular valvar complex.
Arterial
root
Valvar
leaflets
Virtual proximal ring
Sup.
Base
Apex
Inf.
the roots, the distal margins of the

semilunar hinges of the leaets are
attached at the sinutubular junctions
(Figures 3.4, 3.5).
Fig. 3.2 The close-up view of the mitral valve,
Fig. 3.3 A comparable close-up of the aortic valve from Figure 3.1
shows the components of the arterial valvar complex, which
extends from the virtual plane at the nadir of the attachments of
the leaets (white dashed line) to the sinutubular junction.
When comparisons are made between

the overall arrangement of the
atrioventricular and arterial valvar
complexes, one fundamental difference is
that the leaets of the atrioventricular

valves are suspended in true annular
fashion at the atrioventricular junctions
(Figure 3.2), whereas in the arterial valves,
54
Circular anatomical ventriculoarterial junction

Distal attachments at sinutubular junction
Fig. 3.4 The ventriculoarterial junction of the
Semilunar hinges
Proximal attachments to infundibulum
right ventricle has been cut anteriorly, and

opened out. The valvar leaets have been
removed, their remnants revealing their initial
semilunar attachments. Note how these hinges
cross the circular anatomical junction between
the muscular infundibulum and the walls of the
pulmonary valvar sinuses (white dashed line).
All the leaets are supported by muscle, which
therefore forms the basal part of each of the
sinuses of Valsalva. The distal attachments,
however, are at the sinutubular junction.

Continuity with mitral valve
Fig. 3.5 The ventriculoarterial junction of the left ventricle has

Proximal muscular attachments
Circular anatomic ventriculoarterial junction
the leaets are hinged in semilunar fashion,

with the attachments crossing the
anatomical ventriculoarterial junctions
(Figures 3.4, 3.5). It is these differences
that underscore the problems currently
existing in the use of the word annulus
when describing the morphology of the
cardiac valves2. Indeed, the fashion in
which the same words have been used in
been opened and splayed in comparable fashion to that in

Figure 3.4, and again the leaets of the aortic valve have been
removed, revealing the semilunar attachments. In the left ventricle,
because of the brous continuity with the aortic leaet of the
mitral valve, only two of the aortic valvar leaets have muscular
support. The semilunar hinges, however, still cross the circular
anatomical ventriculoarterial junction.
different ways to describe the components

of the various valves has led to much of the
confusion surrounding their specic
anatomy, as was evidenced in a recent
questionnaire answered by cardiac
surgeons working in worldwide centres3.
Thus, some consider that, while the
atrioventricular valves possess leaets, the
moving components of the arterial valves
should be considered as cusps. Our

preference is to achieve uniformity by
describing these working parts in both the
atrioventricular and arterial valves as the
leaets2. As we have already discussed, it is
the individual leaets of the arterial valves
that are semilunar, reecting the shape of
the attachments of the hinges. The overall
anatomical complexes guarding the
55
Fig. 3.6 The aortic valve has been photographed from above,
with the leaets in their closed positions. As can be seen, the zones
of apposition between the leaets (red double-headed arrows)
extend from the peripheral sinutubular junction to the valvar
centroid (white circle). In anatomical terms, these zones are the
commissures between the leaets. Conventionally, however, it is
the peripheral attachments (stars) that are usually dened as the
commissures.
ventriculoarterial junctions, therefore, are

best considered as being arterial rather than
semilunar. With regard to the leaets,
nonetheless, it follows that, because of their
semilunar shape, they are supported in
crown-like fashion within the arterial roots.
There is often a degree of brous
thickening that reinforces these
attachments, the brous thickenings being
more obvious in the aortic than the
pulmonary valve (compare Figures 3.4 and
3.5), but becoming more marked in the
pulmonary valve with ageing. When
considered as a unit, the semilunar
attachments are not strictly circular, and
hence not annular. The remnants of the
leaets are often described during surgical
procedures as representing the valvar
annulus3. Echocardiographers, in
contrast, measure the virtual ring marking
the entrance to the valvar complex as the
annulus, and report this dimension to the
surgeon2. This discrepancy must harbour
the potential for confusion4. Consensus is
now growing, therefore, that surgeons
should also consider this virtual ring as
representing the valvar annulus24. It
would be optimal if the entrance to the
roots was simply described as the valvar
diameter, but this might be too much to
hope for.
Further potential difculties arise with
the use of the word commissure. When
used anatomically, a commissure describes

a line of junction between adjacent
structures, as in the eyelids or lips. These
structures, with two moving parts, have a
solitary commissure, which has two ends.
An alternative denition for commissure,
however, is the end-point of a zone of
apposition2. This is the fashion in which
commissure has been used traditionally
when describing the cardiac valves, and
such usage is unlikely to change3. But, in
addition to taking note of the ends of the
zones of apposition, it is also important to
acknowledge the structure of the zones of
apposition themselves, which extend from
the commissures to the valvar centroid4.
There are three such zones of apposition to
be found in the arterial valves (Figure 3.6),
and also in the tricuspid valve. In the mitral
valve, in contrast, there is a solitary zone
of apposition (Figure 3.7).
POSITION AND SUPPORT OF THE

VALVES WITHIN THE HEART
In the left heart, the leaets of the
atrioventricular and arterial valves are in
close proximity to each other within the
atrioventricular and ventriculoarterial
junctions (Figure 3.1), but their hinges are
well separated within the right heart. These
differences, and the overall arrangement of
the four cardiac valves, are best appreciated

by examining the short axis cylindrical
section of the heart (Figure 3.8). The
leaets of three of the valves are in brous
continuity with one another within this
cylinder, although descriptions of a
brous skeleton providing support for the
leaets within the atrioventricular
junctions are grossly exaggerated. The
arterial sinuses of the pulmonary valve are
supported exclusively by ventricular
muscle, specically by the free-standing
right ventricular infundibulum
(Figures 3.3, 3.9). More distally, the valvar
leaets are hinged from the arterial wall
rather than the infundibular muscle, with
the distal attachments being at the
sinutubular junction, where they form the
commissures, a similar arrangement
pertaining for the aortic valve
(Figure 3.10). When considered in
attitudinally appropriate fashion, the
pulmonary valve is the most anterior and
superior valve, the tricuspid valve is the
most inferior, with the mitral valve being
the most posterior of the four. The aortic
valve is centrally located relative to its
neighbours, and is rightwards and
posterior to the pulmonary valve, even
though it guards the exit of the left
ventricle.
The concept of an extensive brous
skeleton supporting the leaets of all four
56
Aortic leaflet
Fig. 3.7 The mitral valve has been

photographed in its closed position, showing
the view obtained from the left atrium. There is
a solitary zone of apposition (white dashed
line) between the aortic and mural leaets, but
conventionally it is the ends of this zone of
apposition (white circles) that are dened as
the valvar commissures. (The photograph was
taken by Dr Van S. Galstyan, from Armenia, and
we thank him for permitting us to use it in
our book.)
Mural leaflet
Pulmonary valve
Aortic valve
Mitral valve
Sup.
Post.
Ant.
Inf.
Tricuspid valve
valves within the muscular cylinder

forming the base of the ventricular mass,
appealing as it may be, is far from reality.
The pulmonary valvar leaets have no
direct relationship to the leaets of the
other valves (Figures 3.9, 3.11). The
leaets of the two atrioventricular valves
have limited brous support within the
atrioventricular junctions, but it is the
Fig. 3.8 This superior view of a cylindrical section of the heart

through the atrioventricular and ventriculoarterial junctions shows
the relationship of all four cardiac valves.
relationships of the aortic leaet of the

mitral valve that underscore the presence of
the greatest brous support within the
cardiac base. This leaet of the mitral valve
is in direct brous continuity with two of
the leaets of the aortic valve, with the
continuity producing the aortic-mitral
curtain which forms the roof of the left
ventricle. The area of brous continuity is
thickened at both its ends, serving to

anchor the aortic-mitral valvar unit to the
ventricular musculature across the short
axis of the left ventricle (Figure 3.11).
These thickenings are called the right and
left brous trigones, respectively. The
right brous trigone is itself continuous
with the membranous septum, with the
conjunction of these two brous
57
Infundibulum
Pulmonary trunk
Aorta
Fig. 3.9 The cut across the heart to replicate the oblique
subcostal echocardiographic section shows the muscular
infundibular sleeve of the right ventricle supporting the leaets
and sinuses of the pulmonary valve. (See also Figure 3.3.)
Base
Right
Left fibrous trigone
Left
Apex
Fig. 3.10 The aortic root has been opened to
Fibrous continuity
Membranous septum
components forming the strongest part of

the brous support. The conjoined
structure is called the central brous body.
The brous area formed by the
membranous septum continues cranially

to the level of the sinutubular junction.
This wall of the aortic root, interposing
between two of the valvar sinuses, is
show the area of the central brous body as

seen from the left ventricle in anatomical
orientation. The body is made up of the areas
of continuity between the membranous
septum and the right brous trigone. Note that
the brous area then extends distally to the
level of the sinutubular junction, forming a
triangle between the superior parts of the
semilunar attachments of the right coronary
and non-coronary leaets of the aortic valve.
one of the interleaet brous triangles

(Figure 3.12).
Included in the central brous body,
therefore, is this interleaet brous
58
Fibrous
continuity
Pulmonary
trunk
Infundibulum
Base
Post.
Ant.
Apex
Fig. 3.11 To produce this picture, the heart has been dissected to
parallel the parasternal long axis echocardiographic plane
(compare with Figure 3.1). The arterial roots, however, have been
left intact in this specimen, as has the mitral valve, albeit with the
arterial roots cleaned to show the free-standing sleeve of
subpulmonary infundibular musculature that lifts the leaets of
the pulmonary valve away from the base of the ventricular mass.
Note the area of brous continuity between the leaets of the
aortic and mitral valves forming part of the roof of the left
ventricle. The ends are thickened to form the right and left brous
trigones.
Removed triangle
**
*
*
*
Right atrium
Base
Post.
Ant.
Apex
Fig. 3.12 To produce this image, the interleaet triangle

between the right and non-coronary aortic leaets has been
removed, and the heart has been photographed from the right
side. The base of the triangle is formed by the interventricular
component of the membranous septum. The white asterisks show
the cut edge of the ventriculoinfundibular fold, which forms the
greater part of the supraventricular crest. The most apical part of
the removed triangle, however, encroaches on the transverse sinus,
showing that this area was initially part of the parietal wall of the
left ventricle.
triangle, which lls the gap between two of

the semilunar hinges of the leaets of the
aortic valve, in this instance the space
between the non-coronary and right
coronary leaets (Figure 3.10). The
location of the brous triangle can best be
perceived from the right side, the brous
tissue forming its oor having been
removed (Figure 3.12). The triangle is seen
to extend superiorly to the level of the
aortic sinutubular junction, thus separating
the most distal part of the left ventricular
cavity from the right side of the transverse
sinus.
Similar small triangles of brous tissue
form the most distal parts of the spaces
between the other leaets of the aortic
valve5. The triangle between the noncoronary and the left coronary leaets is
itself conuent with the area of brous
continuity between the aortic and mitral
valves (Figure 3.13). When the triangle is
removed, and the heart photographed from
behind, it can be seen how the brous
tissue separates the left ventricular outow
tract from the middle part of the transverse
sinus (Figure 3.14). The much smaller
triangle separating the two coronary leaets
of the aortic valve separates the left
ventricular outow tract from the tissue
plane that runs between the aortic root and

the free-standing right ventricular
infundibulum (Figure 3.15). Taken overall,
therefore, the brous components of the
aortic root form a three-pronged crown
extending to the level of the sinutubular
junction (Figure 3.16). The brous tissue
forming the crown-like aortic root
continues into the left atrioventricular
junction, where it supports the hinge of the
mural leaet of the mitral valve
(Figure 3.17). The extent of this support,
however, varies markedly from heart to
heart6. It is rare to nd a complete cord of
brous tissue (Figure 3.18) encircling the
mitral valvar orice. In many places the
hinge of the leaet is supported by a brous
shelf, or in some instances only by the
brofatty tissues of the left atrioventricular
groove, which also serve to insulate the
atrial from the ventricular myocardium.
The arrangement in which the brofatty
tissues insulate the atrial from the
ventricular muscle masses (Figure 3.19) is
the rule around the orice of the tricuspid
valve. It is very rare to nd the valvar
leaets supported by a brous annulus.
Paradoxically, the solitary normal area of
atrioventricular muscular continuity,
namely the penetrating bundle of His,
59
pierces the strongest part of the brous

skeleton, specically through the
atrioventricular component of the
membranous septum. This point is
conveniently marked for the surgeon by the
attachment to the central brous body of
another brous structure, the tendon of
Todaro. This brous cord extends through
the muscle of the right atrial wall, attaching
to the right-sided aspect of the
membranous septum, and forming the
atrial boundary of the triangle of Koch
(Figure 3.20).
It follows from the above discussion that
it is incorrect to speak of either an aortic or
a pulmonary valvar ring in the sense of a
collagenous structure supporting the valvar
leaets. Rather than being hinged in
uniplanar circular fashion, the leaets of
the arterial valves have semilunar
attachments within the arterial roots
(Figures 3.4, 3.5). True uniplanar rings,
two of them, do exist within the structure
of each arterial root, but neither is
conuent with the semilunar attachments
of the leaets, and neither is taken by
surgeons to represent the valvar annulus.
The most obvious anatomical ring, the
anatomical ventriculoarterial junction,
becomes evident only when the valvar
Transverse sinus
Apex of interleaflet triangle
Sup.
Apex
Base
Mitral valve
Inf.
Fig. 3.13 This heart has been sectioned through the interleaet
triangle between the non-coronary and left coronary aortic
leaets, showing how the brous wall interposes between the left
ventricular outow tract and the transverse sinus (white dotted
line).
60
Non-coronary sinus
Left coronary sinus
Left
Post.
Ant.
Tip of interleaflet triangle
Right
Fig. 3.14 In this heart, the tip of the brous triangle between the
left coronary and non-coronary aortic valvar leaets has been
removed, and the heart photographed from the transverse sinus to
show the location of the apex of the triangle.
Right coronary artery

Tip of interleaflet triangle
Base
Left
Right
Apex
Septal perforating arteries
leaets themselves have been removed,

being best seen in the pulmonary valve
(Figure 3.4). The semilunar attachments of
the leaets cross this ring so that the upper
extremity of each leaet is attached to
another obvious anatomical ring, namely
the sinutubular junction (Figure 3.3).
Fig. 3.15 This dissection shows the location of the interleaet

brous triangle between the two sinuses of the aortic valve that
give rise to the coronary arteries. The triangle itself has been
removed, as has the free-standing sleeve of subpulmonary
infundibulum, and the heart is photographed from the right
ventricular aspect. Note the position of the septal perforating
arteries.
There is a third ring within the arterial

roots, but this one is a virtual rather than an
anatomical structure. It is formed by
joining together the most proximal parts of
the valvar hinges (Figure 3.3). The virtual
ring, however, has particular clinical
signicance, as this is the ring identied by
echocardiographers as the valvar annulus2.

As now suggested by the German Working
Group concerned with surgical treatment
of the aortic valve3, the overall situation
would become much clearer if surgeons
also accepted this virtual ring as
representing the valvar annulus.
61
Central fibrous body
Fig. 3.16 This heart has been dissected by removing the entirety
Sup.
of the atrial myocardium, and is photographed from behind. The

walls of the aortic sinuses have been removed to the level of the
semilunar hingepoints of the valvar leaets, showing the overall
crown-like arrangement of the brous part of the root supporting
the leaets. Note also the brous extensions extending from the
brous tissue of the aortic root into the orices of the mitral and
tricuspid valves. These are the brous components of the annuluses
of the atrioventricular valves.
Membranous septum
Right
Left
Inf.
Aorticmitral continuity
Aortic root
Mitral valvar orifice
Mural leaflet
BASIC MORPHOLOGY OF THE

ATRIOVENTRICULAR VALVES
The mitral and tricuspid valves, as we have
seen, have several important features in
common, which permit their collective

description in terms of a valvar complex
(Figure 3.1)1. The rst feature is the
attachments of the valvar leaets to the
atrioventricular junctions. As discussed
Fig. 3.17 The brous skeleton supporting the

aorticmitral unit has been removed from the
heart shown in Fig. 3.16, and is photographed
from beneath. The brous tissue extends
around the orice of the mitral valve, albeit
that histological studies show variability in the
rmness of the brous support. There is
minimal extension, however, around the
orice of the tricuspid valve (stars). The red
dotted line and the white triangles show the
continuity between the leaets of the aortic
and mitral valves.
earlier, this is appropriately termed the

annulus of the atrioventricular valves,
albeit that it is not always a rm and
continuous collagenous cord. A second
feature is the arrangement of the leaets.
62
Atrial myocardium
Fibrous annulus
Mitral valvar leaflet
Ventricular myocardium
Fig. 3.18 This histological section is through the mural leaet of

the mitral valve. Note that, at this particular part of the junction,
there is a cord-like annulus anchoring the leaet at the
atrioventricular junction. It is the exception rather than the rule,
however, to nd such cords supporting the full length of the mural
leaet6.
Atrial myocardium
Fibroadipose tissue
Tricuspid valvar leaflet
Histologically, these structures have a

spongy atrial and a brous ventricular
layer. The atrial myocardium inserts for
varying distances between the
endocardium and the spongy layer. On rare
occasions, fronds of ventricular
Fig. 3.19 The histological section across the right

atrioventricular junction shows that it is the brofatty tissue of the
atrioventricular groove that, at all points except the penetration of
the bundle of His, insulates the right atrial from the right
ventricular myocardium. There is no brous annulus supporting
the hinge of the tricuspid valve.
musculature may extend into the brous

layer. In normal valves, blood vessels are
found only within the segment of leaet
containing muscular fronds. The leaets
are supported by the tendinous cords,
which themselves insert into the papillary
muscles, or directly into the ventricular

myocardium. These cordal attachments are
the third feature common to both tricuspid
and mitral valves, although there are
fundamental differences in the way each of
the cordal units is arranged. This is also the
63
Hinge of tricuspid valve
Fig. 3.20 This picture, taken in the operating

room, shows how the continuation of the
Eustachian valve, inserting as the tendon of
Todaro into the membranous septum (white
dashed circle), demarcates the atrial border of
the triangle of Koch. The star marks the site of
the atrioventricular node, located at the apex
of the triangle.
Tendon of Todaro
case with the fourth feature, namely the

papillary muscles. The differences in these
various features readily permit the
morphological differentiation of the valves.
Before describing these differences, it is
important to emphasise again those
features of the leaets and their tension
apparatus that are common to both valves.
Considered as a whole, the leaets form
a continuous skirt that hangs from the
atrioventricular junction. The skirt itself is
divided into discrete components, with
these individual sections representing the
individual leaets. Sadly, there is no
current consensus as to how many leaets
there are in each valve, nor as to what
constitutes the point of separation of one
leaet from the other. Traditionally, the
tricuspid valve, as indicated by its name,
has been considered to have three leaets.
Some have argued that the valve has only
two leaets7, albeit that our studies lend no
support to this concept8. The alternative
name for the mitral valve is the bicuspid
valve. Yet it has been suggested that this
valve is best considered as having four9, or
even six leaets10. These ongoing
arguments illustrate the difculties that
exist in dening individual leaets. Some
have sought to achieve such denition by

identifying the so-called commissures
between them. This necessitates dening a
commissure. Surgeons usually take the
commissures as the peripheral attachments
of a breach in the skirt of valvar tissue. For
the atrioventricular valves, these breaches
are supported by fan-shaped commissural
cords, such cords then inserting into a
major papillary muscle or papillary muscle
group (Figure 3.21). This concept depends
on dening one variable structure in terms
of another, which is itself variable. In terms
of philosophy, this is a poor principle. So as
to dene commissures in this fashion, the
valve is also assessed in the opened
position. Yet, as all surgeons know, in order
to function properly, the leaets of the
valve must t together snugly when closed.
Because of this, we prefer to dene the
extent of the leaets according to the
location of the zones of apposition between
them. It is thus the peripheral extents of
these zones of apposition that become the
commissures, and the areas of the skirt
between them that represent the leaets.
The leaets themselves are supported by
tendinous cords. The ends of the zones of
apposition between the adjacent leaets are
usually supported by fan-shaped cords, and

these are dened as commissural cords
(Figure 3.21). In addition to the fan-shaped
cord, the entire free edges of the leaets are
normally uniformly supported by cords
(Figure 3.22). As was indicated by Frater11
when discussing the exquisitely complex
categorisation of cords proposed by the
group from Toronto12, if any of these cords
supporting the free edge are cut, the valve
may become regurgitant. We agree with
Frater11 that, for the purposes of
categorisation, it is sufcient simply to
distinguish those cords supporting the free
edge (Figure 3.22) from those supporting
the rough zone (Figure 3.23). The detailed
classication proposed by the Toronto
group12 has little, if any, surgical utility.
The cords from the rough zone are
prominent structures that extend from the
papillary muscles to the ventricular aspect
of the leaets. Some of the cords to the
rough zone are particularly prominent in
the mitral valve, and are called strut cords
(Figure 3.23). There is a third type of cord
that extends from the ventricular wall close
to the atrioventricular junction, and again
inserts into the ventricular aspect of the
leaet. These are the basal cords
64
Ant.
Sup.
Inf.
Fan-shaped
cord
Aortic leaflet of
mitral valve
Post.
Fig. 3.21 This surgical view of a rheumatic

mitral valve, taken through a left atriotomy,
shows a fan-shaped cord arising from the tip of
its papillary muscle. This is the type of cord
traditionally considered to represent a
commissural cord.
Mural leaflet of
mitral valve
Sup.
Left
Right
Inf.
Aortic leaflet of
mitral valve
Uniform cordal support to free edge
(Figure 3.24). There is little point in

characterising the pattern of branching and
the generations of these cords further, as
long as it is appreciated that, normally, all
parts of the leaets receive good cordal
support. Lack of uniform cordal support is
Fig. 3.22 This anatomical specimen, viewed from the inlet aspect,
shows the aortic leaet of the mitral valve, with tendinous cords
supporting the entirety of the free edge of the valve.
very likely a mechanism leading to prolapse

of a leaet13,14.
The different components of the
atrioventricular valve making up the overall
valve complex (Figure 3.1) must function
in concert so as to produce a competent
valvar mechanism. The reason that the

atrioventricular valves have such a complex
tension apparatus is that, while in their
closed position, they must withstand the
full brunt of systolic ventricular pressure.
A lesion of any of the valvar components
Base
Right
65
Aortic leaflet
of mitral valve
Left
Apex
Fig. 3.23 Some cords to the rough zone of the ventricular
Strut
cords
aspect of the aortic leaet of the mitral valve are particularly

prominent, as shown in this illustration, and are dened as
strut cords.
Fig. 3.24 The mural leaet of the mitral valve has been
reected upwards, showing the basal cords (arrow), each with
their own muscular belly.
can result in regurgitation. Thus, it is

essential that the atrioventricular junction
be of normal size and not overly dilated.
The leaets must coapt snugly. This
demands an area of overlap. Often,

particularly in aged valves, this point of
closure is marked by a series of nodules.
The point of closure away from the free
edge gives a margin of safety for dilation of

the valvar orice. Equally signicant to
competent closure, however, is the support
of the leaet by cords attached to the free
66
edge. As discussed previously, this feature

is highly signicant to the mechanism of
prolapse of the valve leaets. It has been
suggested15 that prolapse of the leaets of
the mitral valve is one of the most common
congenital lesions. As yet, there is no
consensus as to the aetiology of such
prolapse. Morphological observations11,12
certainly suggest that lack of cordal support
to the free edge of the leaets can
contribute to prolapse, along with
lengthening of the persisting cords;
uniform support of the free edges of the
leaets is an integral part of a normal valve.
Also important in the maintenance of
competence is the correct action of the
papillary muscles and the ventricular
myocardium. Not only must the papillary
muscles be viable, but they must also be in
their appropriate position of mechanical
advantage relative to the axis of the valve.
Taken together, all parts of the valvar
unit are signicant in the normal function
of the valve1.
THE MITRAL VALVE

The mitral valve has two major leaets,
which are supported by paired papillary
muscles. The two leaets have widely
dissimilar circumferential lengths
(Figure 3.25). The ends of the solitary zone

of apposition between them, traditionally
labelled as the commissures, together with
the papillary muscles, are positioned in the
inferoanterior position adjacent to the
septum, and superoposteriorly arising from
the posterior ventricular wall, although
they are usually described incorrectly as
being posteroseptal and anterolateral. The
advent of computed tomographic imaging,
which relates the location of the heart to the
coordinates of the body, reveals the
inappropriate nature of the current
description (Figure 3.26). The
tomographic images also clearly show the
angle existing between the axis of opening
of the valve and the plane of the inlet
component of the muscular ventricular
septum (Figure 3.26). The posterior
extension of the subaortic outow tract is
wedged into this angle. As we will see, this
arrangement is key to the disposition of the
axis of atrioventricular conduction tissue
(see Chapter 5). The two leaets of the
mitral valves themselves are frequently
described as being anterior and
posterior. Strictly speaking, they are
anterosuperior and posteroinferior, again
because of the oblique axis of opening of
the valve relative to the anatomical axes of
the body (Figure 3.26). We prefer to
describe the leaets as being aortic and
mural, this description accounting for both

their morphology and their usual location
within the body.
The aortic leaet is attached to only
one-third of the annular circumference. It
is trapezoidal, or more semicircular, in
shape than the mural leaet (Figure 3.27).
The mural leaet, attached to two-thirds of
the annulus, is a long, rectangular
structure, although its middle component
can also be somewhat semicircular. It is the
segments of the mural leaet adjacent to the
ends of the zone of apposition with the
aortic leaet that show the most variation in
morphology. Often these segments are
almost completely separate from the central
component, producing the so-called
scallops of the mural leaet. Carpentier16
has coded these scallops in alphanumeric
fashion, along with the facing segments of
the aortic leaet, and this convention has
widespread surgical use (Figure 3.28).
Yacoub9 has gone so far as to nominate the
scallops as separate leaets of a
quadrifoliate valve. Others10 point out that
the parts of the leaet adjacent to the ends
of the zone of apposition can also take a
scallop-like appearance (Figure 3.29). All
these categorisations are articial. Because
of the marked variability that occurs
normally in the valve, the mural leaet can
be comprised of four, ve, or even more
Fig. 3.25 The mitral valve is viewed from

above in a closed position, as it might be seen
by the surgeon. There is a solitary zone of
apposition between its two major leaets, with
slits in the mural leaet producing a number of
potential subcomponents in the mural leaet
(stars). Note that the ends of the zone of
apposition do not extend to the
atrioventricular junction (bold red braces). (The
original photograph of the mitral valve was
kindly sent to us by Dr Van S. Galstyan, from
Armenia, and we thank him for permitting us
to use it in our book.)
67
Superoposterior
papillary muscle
Subaortic
outflow tract
Fig. 3.26 The computed tomographic image clearly shows the

Left ventricle
Right
ventricle
Inferoanterior
papillary muscle
orientation of the short axis of the ventricular mass relative to the

bodily coordinates, indicated both by the box seen to the bottom
right-hand corner of the image, and the yellow abbreviations at the
margins of the image showing the coordinates of the head (H), feet
(F), anterior (A), and posterior (P). The tomographic section clearly
shows the angle between the line of the zone of apposition
between the leaets of the mitral valve and the plane of the
inferior part of the muscular ventricular septum (white dotted
lines). Note that, contrary to the usual descriptions, the papillary
muscles are positioned inferoanteriorly and superoposteriorly
when considered in attitudinally correct orientation (see
orientation box).
Base
Left
Aortic leaflet
Right
Apex
Mural leaflet
Fig. 3.27 Opening the mitral valve through the end of the zone
of apposition between the leaets closest to the septum, and
spreading the valve, illustrates the widely dissimilar lengths of the
two leaets at their hingepoints from the atrioventricular junction.
components. It is better to recognise that

there are a variable number of slits in the
mural leaet, and that these slits act as
pleats so that the normal valve can close
snugly17. In the setting of prolapse,
nonetheless, individual components of
the divided mural leaet may be deformed
in isolation, and must then be recognised
as such.
The supporting cords attach the leaets

either to the papillary muscles, in the form
of the cords to the free edge and rough
zone, or directly to the wall of the ventricle,
as with the basal cords. The cords at the
ends of the zone of apposition attach
adjacent sides of both the leaets to the
apex of each of the papillary muscles.
Supplementary heads of both papillary
muscles then support other cords running

to the free edge, which usually attach
uniformly along the leading edge of the
leaets, although, as we have discussed, the
degree of support can be quite variable.
The cords supporting the divisions
between the scallops of the mural
leaet also often have a fan-shaped
appearance. When attached to a prominent
68
A1
A2
A3
P1
P2
P3
Fig. 3.28 The classication of Carpentier16 recognises three

major scallops in the mural leaet (P1 to P3), and also names the
facing segments of the aortic leaet in alphanumeric fashion (A1
to A3). This classication, however, ignores the potential
subcomponents of the leaet at the ends of the solitary zone of
apposition (see Figure 3.29).
1
2
A1
A2
A3
P1
3
P2
P3
5
supplementary head of a papillary muscle,

they can be markedly similar to the cords
supporting the commissures. The cords to
the rough zone run from the papillary
muscles to the ventricular aspect of the
leaets. As already emphasised, several of
these cords, running from each papillary
muscle to the undersurface of the aortic
leaet, are particularly prominent, and are
called strut cords (Figure 3.23). The basal
cords attach to the ventricular aspect of the
mural leaet, running directly from
miniature muscle heads on the parietal wall
of the ventricle (Figure 3.24).
The papillary muscles of the mitral
valve are almost always prominent paired
Fig. 3.29 Kumar and colleagues10, in their suggested

classication, also took into account the components of the mural
leaet adjacent to the ends of the solitary zone of apposition
between the two major leaets (stars), giving six rather than four
potential subcomponents for the entire valvar skirt (compare with
Figure 3.28).
structures, located on the inferoanterior

and superoposterior aspects of the parietal
ventricular wall (Figure 3.26). Although
there can be considerable variation in the
precise morphology of either muscle13,
neither arises from the septum, in contrast
to the attachments of the tricuspid valve
within the right ventricle. When the valve
is dissected in its natural position within
the left ventricle (Figure 3.30), the muscles
are seen to be adjacent to each other at the
junction of the apical and middle thirds of
the parietal ventricular wall.
It is the area of the valvar orice related
to the right brous trigone and central
brous body that is most vulnerable in
terms of the conduction tissues. The

atrioventricular node and the penetrating
atrioventricular bundle are adjacent to
these areas (Figures 3.31, 3.32). The area of
the orice between the two trigones, more
or less the midportion of the aortic leaet,
is directly related to the commissure
between the non-coronary and left
coronary leaets of the aortic valve. At this
point, the aortic root is tented up, and an
incision apparently through the atrial wall
will extend into the subaortic outow tract.
If the incision is continued superiorly, it
will pass into the transverse sinus of the
pericardial cavity, which overlies the
aortic-mitral curtain (Figure 3.13). The
Posterosuperior muscle
69
Sup.
Base
Apex
Inf.
Fig. 3.30 The parietal wall of the left

ventricle has been removed and the heart
photographed from behind and beneath. The
papillary muscles are directly adjacent at their
ventricular origins.
Anteroinferior muscle
Excised non-coronary aortic sinus

Location of AV node
Mitral
valve
Tricuspid
valve
Ant.
Left
Right
Post.
Fig. 3.31 The atrial chambers have been removed, along with the
non-adjacent sinus and leaet of the aortic valve, revealing the
deep posteroinferior diverticulum of the left ventricular outow
tract. Note the position of the atrioventricular (AV) node (red circle)
relative to the mitral valve.
70
Site of atrioventricular node

Coronary sinus
Sinus node
Fig. 3.32 The cartoon shows the components of the mitral valve
Dominant right
coronary artery
Circumflex artery
deep inner curvature of the heart overlies

and runs to either side of the curtain, and is
lined by the transverse sinus. Encircling
the mural leaet of the valve are the
circumex coronary artery from below and
to the left, and the coronary sinus from
below and to the right (Figure 3.32). The
atrioventricular nodal artery also runs in
close proximity to the right side of the
mitral orice, particularly when arising
from a dominant circumex artery. Indeed,
the extent of the margin directly related to
the circumex artery depends on coronary
arterial disposition. When the left coronary
artery is dominant, an arrangement found
in one-tenth of the population, the entire
attachment of the mural leaet is intimately
related to the coronary artery, including its
branch supplying the atrioventricular (AV)
node (Figure 3.33).
THE TRICUSPID VALVE

As judged on the basis of its pattern of
closure8, the tricuspid valve has three
major leaets (Figures 3.34, 3.35).They are
positioned septally, anterosuperiorly, and
inferiorly (Figure 3.36), albeit that the
relative to the structures of surgical signicance when the right

coronary artery is dominant, this being the situation in 90% of
individuals.
inferior, or mural, leaet is usually

described as being posterior. This is yet
another example of the description of
cardiac structures as seen in the autopsy or
dissecting room, the heart having been
removed, rather than using the bodily
coordinates for reference. The septal leaet
is supported between the relatively
inconstant inferior papillary muscle, and
the much more constant medial papillary
muscle. Also known as the muscle of
Lancisi, or the papillary muscle of the
conus, it arises from the posterior limb of
the septomarginal trabeculation
(Figure 3.37). The attachment of the septal
leaet crosses the membranous septum,
dividing it into atrioventricular and
interventricular components (Figure 3.38).
The distal extent of the leaet is attached
by multiple cords running from the free
edge directly to the septum (Figure 3.39).
It is this feature that serves to
distinguish the tendinous support of the
tricuspid valve from that of the mitral
valve, the mitral valvar leaets lacking any
septal attachments. In the area where the
septal leaet extends across the
membranous septum, the leaet may be
divided, resulting in a discrete cleft

that extends towards the central
brous body.
The anterosuperior leaet descends from
the underside of the ventriculoinfundibular
fold, hanging like an extensive curtain
between the inlet and outlet parts of the
ventricle. Its medial and superior end is
supported by cords from the medial
papillary muscle (Figure 3.37). There is
more variability in the arrangement of its
lateral and inferior component. On occasion,
its zone of apposition with the inferior leaet
is supported by the prominent anterior
papillary muscle, which takes origin from
the apical portion of the septomarginal
trabeculation. More often, the anterior
muscle supports the midportion of the
anterosuperior leaet. The leaet is well
supported by cords to its free edge, but also
by rough-zone and strut cords, in a fashion
comparable with that seen in the aortic
leaet of the mitral valve. When the anterior
papillary muscle supports the midpoint of
the anterosuperior leaet, the muscle
supporting its zone of apposition with the
inferior leaet is relatively indistinct.
Frequently, there are extensions of
71
Ant.
Right
Left
Post.
Fig. 3.33 The dissection, photographed from
Artery to AV node
Circumflex artery
Inferior interventricular artery
above and behind, with the heart in the

anatomical position, shows the relationship of
the circumex artery to the mural leaet of the
mitral valve when the left coronary artery is
dominant. Note that the inferior
interventricular artery, and the artery to the
atrioventricular (AV) node, take their origin
from the circumex artery.
Inferior
leaflet
Ant.
Inf.
Sup.
Septal leaflet
Post.
muscle from either the papillary muscles or

the ventriculoinfundibular fold directly
into the anterosuperior leaet. The third
leaet of the tricuspid valve is the inferior or
Fig. 3.34 This picture, taken in the operating room, shows the
arrangement of the three leaets of the tricuspid valve, which are
positioned septally, anterosuperiorly, and inferiorly or murally. The
latter leaet is usually described, incorrectly, as being positioned
posteriorly.
mural one. It is less constant than the

other two, as the inferior papillary muscle
is often indistinct and variable. The leaet
is attached to the parietal part of the
atrioventricular junction, being

supported distally by several cords to
the free edge, attached either to small
heads of muscle, or directly into the
72
Anterosuperior
leaflet
Inferior
leaflet
Septal
leaflet
Fig. 3.35 The anatomical specimen has been

Sup.
photographed to replicate the arrangement as

seen in the operating room, as shown in
Figure 3.34, conrming that the leaets are
located septally, anterosuperiorly, and
inferiorly. Note the relationship of the septal
leaet to the landmarks of the triangle of Koch
(white dotted lines).
Base
Triangle
of Koch
Apex
Inf.
Anterosuperior
leaflet
Septal
leaflet
Fig. 3.36 The computed tomographic image, showing the three

leaets of the tricuspid valve, conrms that they are positioned
septally, anterosuperiorly, and inferiorly when the heart is viewed
in attitudinally appropriate fashion. Note the markers produced by
the computed tomographic software.
Inferior
leaflet
ventricular wall. There are usually also basal

cords supporting the inferior leaet
(Figure 3.40).
The entire parietal attachments of
the leaets of the tricuspid valve are
encircled by the right coronary artery

running in the atrioventricular groove
(Figure 3.41). It is rare to nd a
well-formed collagenous tricuspid
annulus. Instead, the atrial and ventricular
myocardial masses are separated almost

exclusively by the adipose tissue within the
groove, with the leaets of the valve hinged
from the endocardial surface of the
ventricular wall (Figure 3.20).
Sup.
73

Left
Right
Inf.
Fig. 3.37 The right ventricle is photographed from the
Antero-superior
leaflet
Inferior leaflet
Septal leaflet
Septomarginal
trabeculation
infundibular aspect in anatomical orientation, showing the medial

papillary muscle, also known as the muscle of Lancisi, supporting
the zone of apposition between the septal and the anterosuperior
leaets.
Aortic root
Mitral
valve
Tricuspid
valve
Sup.
Right
Left
Inf.
Interventricular membranous septum
BASIC ARRANGEMENT OF THE

ARTERIAL VALVES
The arterial valves, although much simpler
structures than the atrioventricular valves, as
Fig. 3.38 The base of the heart is shown from the atrial aspect,
the atrial myocardium and the non-coronary sinus of the aortic
valve having been removed. The attachment of the septal leaet of
the tricuspid valve (arrow) divides the membranous septum into its
atrioventricular and interventricular components.
they do not need an intricate arrangement of

tension apparatus to ensure their
competence, are still best analysed in terms
of a valvar complex (Figure 3.3). Their
design is simplicity itself. The three
semilunar leaets open into the supporting

arterial sinuses during ventricular systole,
and then collapse together during diastole,
being held in their closed position by the
hydrostatic pressure of the column of blood
74
Septal leaflet
Ant.
Oval fossa
defect
Inf.
Sup.
Post.
cordal attachments (arrows) to the septum of the septal leaet of
the tricuspid valve. The patient also has a defect in the oval fossa.
Sup.
Left
Right
Inf.
Fig. 3.40 The inferior leaet of the tricuspid valve has been
reected away from the ventricular wall to show the basal cords
(arrows).
they support (Figure 3.42). In terms of

histological structure, the leaets have a
brous core with an endothelial lining
(Figure 3.43). The brous core is thickened
at the free edge, particularly at the central
portion short of the free edge where, with
age, a distinct brous thickening, the nodule
of Arantius, becomes evident. The leaets of
the valve do not close at their free edge. The

line of closure is some distance from the free
edge, towards the semilunar attachment. Not
infrequently, the leaets may be perforated
beyond the line of closure in this outer
component. Such perforations are a normal
nding, and do not affect valvar function. It
is the hinge line of the individual leaets that
is of half-moon, or semilunar, shape

(Figures 3.4, 3.5). When the three semilunar
hinges are considered in terms of their
overall structure, the seating of the leaets
takes the form of a coronet (Figure 3.44).
The attachments of the leaets at the apex of
their zones of apposition are signicantly
higher than the attachments at their
Ant.
75
Right
Left
Post.
Tricuspid valvar orifice
Fig. 3.41 This view of the heart, also shown in

Figure 3.66, with a dominant right coronary artery,
photographed in anatomical orientation from above
and behind, shows the intimate relationship of the
artery to the orice of the tricuspid valve.
Artery to atrioventricular node
Sup.
Left
Right
Inf.
Fig. 3.42 This image of the closed aortic valve from above shows
the three valvar leaets (1, 2, 3) coapting snugly, held together by
the diastolic pressure of the column of blood they support. The stars
mark the commissures, which are the peripheral attachments of the
zones of apposition to the sinutubular junction.
midportion. In light of these arrangements,

it is less than ideal to describe the hinges of
the valvar leaets in terms of the annulus,
albeit that many surgeons still consider this
to be an appropriate term3. There are two
true rings within the extent of the arterial

root, but paradoxically neither has attracted
attention from surgeons for description as
the valvar annulus. Perhaps the most
obvious is the sinutubular junction, this
being the plane of attachment of the valvar

commissures (Figure 3.45). The most
obvious ring anatomically is the locus over
which the brous walls of the arterial trunks
are attached to the supporting ventricular
76
Leaflet
Wall of aorta
Ventriculoarterial
junction
Fig. 3.43 The histological section shows the brous core of one leaet
of the aortic valves, with its endothelial linings on the arterial and
ventricular aspects. Note that the valvar hinge is well below the
anatomical ventriculoarterial junction.
Valvar hinge
Sup.
Crown-like configuration
Right
Left
Inf.
Fig. 3.44 The aortic root in this specimen has been dissected by
Mitral valvar orifice
removing the valvar sinuses, leaving behind the semilunar

attachments of the leaets. It shows how the overall arrangement
is crown-like.
77
Fig. 3.45 The cartoon shows the relationship of the semilunar

hinges of the aortic valvar leaets (yellow) relative to the rings
within the root. The superiorly located green ring is the sinutubular
junction. The blue ring shows the ventriculoarterial junction, while
the red ring is a virtual structure, formed by joining together the
proximal attachments of the valvar hinges.
Fig. 3.46 The cartoon shows the plane of space measured by

echocardiographers as representing the arterial valvar annulus. It is
the virtual plane constructed by joining together the nadirs of the
hinges of the leaets (blue double-headed arrow). It is a virtual
anatomical ring, however, rather than a true anatomical entity.
The anatomical rings in the arterial roots are formed by the
sinutubular junction (red double-headed arrow), and the
anatomical ventriculoarterial junction (plane indicated by dotted
line). Note that the widest part of the root is at the midsinusal level
(green double-headed arrow).
structures, thus constituting the

anatomical ventriculoarterial junction, and
best seen in the right ventricular outow
tract with the valvar leaets having been
removed (Figure 3.4). There is a marked
discrepancy between this ring-like
anatomical junction and the
haemodynamic junction, the latter

being marked by the semilunar attachment
of the leaets. By virtue of this
arrangement, part of the arterial wall,
haemodynamically, is a ventricular
structure. Part of the ventricle at the base
of each sinus is, in contrast, within the
arterial trunk (Figure 3.46). Thus, despite

the simple nature of the valvar structure,
the overall arterial roots are complex
structures. The sinuses themselves are
arranged in clover-like fashion, permitting
the valvar leaets to close in the fashion of
a Mercedes-Benz sign (Figure 3.42). It is
78
the nadirs of attachment of the valvar

leaets, however, that are taken as the
point of measurement of the
echocardiographic valvar annulus
(Figure 3.46). This plane, therefore, does
not correspond to any anatomical
structure, albeit that consensus among
surgeons is now moving towards accepting
it as representing the valvar annulus3. In
reality, it is no more than the diameter of

the entrance to the arterial roots.
THE AORTIC VALVE

The semilunar leaets of the aortic valve
are attached in part to the area of brous
continuity with the aortic leaet of the
mitral valve, and in part to the muscular
outlet portion of the left ventricle

(Figure 3.47). When naming the leaets of
the valve, advantage can be taken of the fact
that, almost without exception, the major
coronary arteries take origin from two of
the aortic sinuses, but not the third. Thus,
the aortic leaets can accurately be
described as being right coronary, left
coronary, and non-coronary (Figure 3.48).
Sup.
Right
Left
Inf.
Fig. 3.47 The aortic root has been displayed by removing the
leaets of the aortic valve, the outow tract between the left
coronary and the non-adjacent aortic leaets having been opened.
The two sinuses giving rise to the coronary arteries have septal
musculature at their bases (inner red arrows), while part of the left
coronary sinus, along with the non-coronary sinus, is supported by
a brous continuity with the aortic leaet of the mitral valve (outer
red arrows).
Left ventricle

Pulmonary valve
Right coronary
aortic sinus
Left coronary
aortic sinus
Fig. 3.48 The heart has been photographed from above, the
Sup.
Right
Left
Non-adjacent
sinus
Inf.
tubular aorta having been removed to reveal the valvar sinuses.

The origins of the coronary arteries permit two sinuses to be named
as right and left coronary aortic sinuses. The third sinus is nonadjacent relative to the pulmonary trunk. In almost all instances, it
does not give rise to a coronary artery. In those circumstances, it can
also be called the non-coronary sinus.
79
Fig. 3.49 The image is an enlargement of the heart shown in

Figure 3.47. It shows how the bases of the sinuses supporting the
coronary aortic leaets are made up of the musculature of the
ventricular septum (dotted black lines), because the semilunar
hinges extend apically beyond the anatomical ventriculoarterial
junction. The red triangle enclosing a white cross-hatched area
shows the region of aortic wall that forms the distal extent of the
outow tract between the coronary aortic leaets, running to the
level of the sinutubular junction.
However, because the so-called noncoronary sinus can rarely give rise to one of
the coronary arteries, it can more accurately
be described as the non-adjacent sinus. It is
the right and left coronary aortic leaets
that have a predominantly muscular origin
from the left ventricular wall, the base of
the myocardium being incorporated within
the supporting aortic sinus (Figure 3.49).
These are the leaets that are adjacent to
the pulmonary trunk. Their more distal
adjacent parts take origin from the free
aortic wall, with the small interleaet
brous triangle between them separating
the cavity of the outow tract from the
tissue plane between the aortic root and the
subpulmonary infundibulum
(Figures 3.15, 3.50).
As the attachment of the right coronary
leaet is traced from its zone of apposition
with the left coronary leaet, it drops
towards the crest of the muscular part of
the septum in the area of the membranous
septum. It then rises again to the apex of
the zone of apposition with the nonadjacent leaet (Figure 3.51). The
attachment of this posterior part of the
right coronary leaet is an integral part of
the brous skeleton. All of the nonadjacent leaet has a brous origin, and is
incorporated in the brous skeleton. Half

of it, extending from the zone of apposition
with the right coronary leaet, is attached
to the area of the membranous septum
(Figure 3.51). The triangle between these
leaets has important relationships to both
right atrium and right ventricle
(Figure 3.52). The extensive posterior
extension, or diverticulum, of the left
ventricular outow tract lies beneath the
non-adjacent leaet, limited anterolaterally
by the atrioventricular component of the
membranous septum. Reaching its nadir at
the attachment to the right brous trigone,
the non-adjacent leaet then rises to the
apex of its zone of apposition with the left
coronary leaet. The adjacent parts of both
leaets in this area are attached to the free
aortic wall distally, and proximally are
continuous with the aortic leaet of the
mitral valve. In this way, they form the
aortic-mitral curtain, which separates the
left ventricular outow tract not from the
left atrium, but from the transverse sinus of
the pericardium (Figure 3.53). Beyond this
area, the left coronary leaet adjoins a short
segment of parietal myocardium before
extending anteriorly to reach the zone of
apposition with the right coronary leaet.
The precise attachments of the aortic
leaets vary from heart to heart,

particularly with regard to that portion of
the zone of apposition between the noncoronary and left coronary leaets that is
related to the aortic-mitral curtain. The
length of the subaortic brous curtain also
varies from heart to heart. In a small
percentage of normal hearts, the aortic
valve in this area is supported by a complete
muscular infundibulum or sleeve18. These
individual variations do not distort the
basic anatomical relationships as described
earlier.
Appreciation of the anatomy of the
valvar hinges brings into focus the
important surgical danger areas related to
the aortic valve. The ascending part of the
non-coronary aortic leaet is positioned
directly above the part of the atrial wall
containing the atrioventricular node, while
the zone of apposition with the right
coronary aortic leaet is above the
penetrating atrioventricular bundle and the
membranous septum (Figures 3.54, 3.55).
The zone of apposition between the right
coronary and left coronary leaets is
usually positioned opposite the
corresponding zone of apposition between
the facing leaets of the pulmonary valve.
The adjacent parts of the two aortic
80
Pulmonary trunk
Infundibulum
Fig. 3.50 The aortic root in this heart has been dissected from the
Base
Left
Right
Left coronary aortic leaflet
Apex
right side, showing the relationship with the free-standing

muscular subpulmonary infundibulum (white brace). The root has
been cut along the brous triangle separating the two coronary
aortic leaets (white arrow), showing how this triangle separates
the aortic outow tract from the tissue plane between the aortic
root and the infundibulum (star).
Non-coronary
aortic sinus
Right coronary
aortic sinus
Aortic leaflet of mitral valve
coronary leaets, therefore, are directly

related to the infundibulum of the right
ventricle, albeit that a discrete extracardiac
tissue plane interposes between them
(Fig. 3.50). Incisions through the right
Fig. 3.51 The aortic root shown in Figure 3.50 has been enlarged
to show the triangle between the right coronary aortic leaet and
the non-adjacent leaet. The base of the triangle is made up of the
membranous septum (dashed white oval), while the apex of the
triangle rises to the sinutubular junction (red triangle). The white
arrow shows the continuity with the aortic leaet of the mitral
valve, and the black asterisk indicates the right brous trigone.
ventricular outow tract at this site lead

directly into the subaortic region
(Figure 3.56). This is the basis of the
right ventricular approach for relief of
subaortic obstruction, as in the Konno, or
modied Konno, procedure. Beyond this

point, the lateral part of the left coronary
leaet is the only part of the aortic valve
that is not intimately related to another
cardiac chamber. This is the part of the
81
Base
Right
Left
Apex
Tricuspid valve
Fig. 3.52 The dissection through the brous triangle between the
right and non-adjacent leaets of the aortic valve shows the
relations of the aortic outow tract to the right atrium (black brace)
and right ventricle. The black dotted line shows the apex of the
triangle, which separates the left ventricular outow tract from the
transverse sinus (white asterisk). The white triangle is between the
non-adjacent and left coronary aortic sinuses.
Transverse sinus
Apex of interleaflet triangle

Left ventricle
Sup.
Base
Apex
Inf.
valve that takes origin from the lateral

margin of the inner heart curvature. It is in
relationship externally with the free
pericardial space.
Fig. 3.53 The left ventricular outow tract in this heart is

bisected through the brous triangle between the non-adjacent
and left coronary aortic leaets. The brous wall (red dotted line)
separates the outow tract from the transverse sinus (white
dotted line).
THE PULMONARY VALVE

The pulmonary valve in the normal heart
has exclusively muscular attachments to
the infundibulum of the right ventricle

(Figure 3.57). Because of its oblique
position, there is difculty in naming the
pulmonary leaets according to the body
82

Infundibulum
Aortic root
Tricuspid valve
coronary aortic sinus and the supporting ventricular septum.

Viewed in surgical orientation, it shows how the atrioventricular
conduction axis (black line) penetrates the membranous septum
(black brace), extending from the atrioventricular node (red
asterisk) to branch on the muscular ventricular septum (blue
dashed lines).
Left bundle branch
Fig. 3.54 The heart has been prepared by removing the right
Right coronary
aortic sinus
Left coronary
aortic sinus
Non-coronary aortic sinus
Fig. 3.55 The cartoon shows the relationships of the
Atrioventricular node
coordinates. It is better to describe them

according to their relationship to the aortic
valve (Figure 3.58). The two leaets of the
aortic valve that are attached to the septum
are always adjacent to two leaets of the
pulmonary valve. These two leaets of the
pulmonary valve, therefore, can simply be
called the right and left adjacent leaets.
The third leaet is then described,
atrioventricular node and penetrating bundle as they would be

seen by the surgeon working through the aortic valve.
appropriately, as the non-adjacent leaet.

When looked at from the stance of the
observer positioned within the nonadjacent sinus, the two adjacent sinuses are
to his or her right- and left-hand sides19.
The right-hand leaet as seen from the
pulmonary trunk, however, is positioned
leftwards and posteriorly in space, while
the left-hand leaet is adjacent to the right
coronary artery. Our preference, therefore,

is to account for their anatomical location.
The zone of apposition between the two
adjacent leaets has traditionally been
considered to be attached to the muscular
outlet septum immediately above the
anterior limb of the septomarginal
trabeculation. In reality, it is attached to a
free-standing sleeve of infundibular
83
Left coronary aortic sinus

Infundibulum
Pulmonary trunk
Fig. 3.56 The cartoon shows how, in the Konno operation, an
Aorta

Right coronary aortic sinus
incision through the posterior wall of the subpulmonary

infundibulum takes the surgeon into the subaortic outow tract,
the incision in the infundibular wall being carried into the muscular
ventricular septum, and entering the left ventricular outow tract
between the two aortic sinuses giving origin to the coronary
arteries. The two stars show how the wall has been folded open.
Pulmonary valve
Muscular infundibulum
Left
Sup.
Inf.
Fig. 3.57 This heart, shown in anatomical orientation, is opened

along the parietal wall of the right ventricle. It shows how all
leaets of the pulmonary valve are supported by infundibular
musculature.
Right
musculature (Figure 3.50). As the right

adjacent leaet drops down from the apex
of its zone of apposition with the other
adjacent leaet, it is supported by the inner
heart curve, which separates it from the
tricuspid valve. This muscular mass is the
supraventricular crest of the right
ventricle. The particular part separating
the hinges of the atrioventricular and

arterial valves is the ventriculoinfundibular
fold. The zone of apposition between the
right and non-adjacent leaets is thus
towards the parietal extent of this fold, with
the non-adjacent leaet being supported by
the anterior parietal wall of the
infundibulum. The zone of apposition
between the non-adjacent and left adjacent

leaets extends from the parietal
attachment back towards the septum, so
that the left adjacent leaet runs from the
parietal wall to the area of the septum.
When considered as a whole, the valve can
be liberated from the right ventricle,
together with its infundibular sleeve
84
Non-adjacent
component
Rightward adjacent
component
Leftward adjacent
component
Developing aortic valve
Developing pulmonary valve
Right coronary
component
Sup.
Left
Right
Non-adjacent
component
Left coronary
component
Inf.
Fig. 3.58 The image shows the components of the developing

aortic and pulmonary roots, as seen in the mouse at 12.5 days of
embryonic life. The cushions will excavate so as to give rise to the
valvar leaets and their supporting sinuses. The components retain
their relationships, so that two of the sinuses of the pulmonary
valve are always adjacent to the sinuses of the aortic valve that,
eventually, will give rise to the coronary arteries. The other sinus is
non-adjacent. This provides a logical means of describing the valvar
sinuses and leaets, as shown by the labels in this gure.
Aortic valve
Left
Post.
Fig. 3.59 The pulmonary trunk has been
Ant.
Right
Infundibular sleeve
(Figure 3.59) without damaging any

vital structures, although the left adjacent
leaet is closely related to the rst
septal perforating artery (Figure 3.60).
Preservation of this artery is one of the
features that underscores the success of the
Ross procedure20.
RELATIONSHIPS OF THE VALVES

TO OTHER VITAL CARDIAC
STRUCTURES
Positioned as they are within the
ventricular base, the tricuspid, mitral, and
aortic valves are intimately related to two
tilted forwards in this heart, showing the sleeve

of infundibular musculature that lifts the
leaets of the pulmonary valve away from the
ventricular base. Note the origin of the rst
septal perforating artery.
vital cardiac subsystems, namely the

atrioventricular conduction system and the
coronary circulation. It is mandatory to
avoid damage to these structures during
surgery, so it is important to know their
precise locations. This must be learned
relative to landmarks within the valves
85
Left
Sup.
Inf.
Right
Fig. 3.60 The subpulmonary infundibulum has been removed

Infundibulum
themselves, as the conduction tissues are

largely invisible, while the course of the
coronary vessels is likely to be hidden as the
surgeon approaches the valves. Although
we have mentioned these landmarks when
discussing the individual valves, their
importance is such that they justify a
collective review.
THE SPECIALISED MUSCULAR

AXIS FOR ATRIOVENTRICULAR
CONDUCTION
In the normal heart, the penetrating
atrioventricular bundle is the only
muscular communication between the
atrial and ventricular muscle masses. The
bundle penetrates the membranous septal
component of the central brous body. It
is, therefore, intimately related to the
leaets of the mitral, tricuspid, and aortic
valves. The axis takes its origin from the
atrioventricular node and its atrial zones of
transitional cells. These atrial components
of the conduction axis are contained
completely from the heart shown in Figure 3.59, revealing how this
can be achieved without transgressing on the left ventricle. Note
again the location of the rst septal perforating artery.
exclusively within the triangle of Koch.

The inferior extent of the triangle is the
junctional attachment of the septal leaet of
the tricuspid valve. As the axis penetrates
the septum, it immediately enters the
subaortic region of the left ventricle
(Figure 3.61). The point of penetration is
related to the anteroinferior end of the zone
of apposition between the leaets of the
mitral valve. In this area, the
atrioventricular node lies within ve to 10
millimetres of the atrial attachment of the
medial scallop of the mural leaet
(Figure 3.32). Having reached the left
ventricular outow tract, the conduction
axis begins to branch, either on the crest of
the muscular ventricular septum,
sandwiched between it and the
interventricular membranous septum
(Figure 3.52), or on the left ventricular
aspect of the septum. This area is
immediately beneath the zone of apposition
between the non-adjacent and right
coronary leaets of the aortic valve
(Figure 3.54), but the height of the
interleaet brous triangle separates the

valvar attachments from the conduction
axis. When seen from the left ventricle, the
left bundle branch fans out as a continuous
sheet on the smooth left septal surface
beneath the zone of apposition, splitting
into its three divisions as it approaches the
ventricular apex. The right bundle branch
takes origin from the axis beyond the takeoff of the left bundle branches. It courses
back across the septum as a thin, insulated
cord, which emerges on the right
ventricular aspect in the area of the medial
papillary muscle. It then runs, usually
intramyocardially, within the structure of
the septomarginal trabeculation, to ramify
at the ventricular apex. When considered
from the right side, the site of the
branching segment of the conduction axis
can be imagined as a line joining the apex of
the triangle of Koch to the medial papillary
muscle (Figure 3.62). Care must be taken
when placing sutures in this area, because
the overall axis is within ve millimetres of
the right ventricular septal surface.
86
Sup.
Excised
membranous
septum
Right
Left
Aorta
Inf.
Fig. 3.61 This dissection is through the membranous septum

(white dotted line). The atrioventricular conduction axis penetrates
this septum to reach the crest of the muscular ventricular septum.
As can be seen, the point of penetration is directly adjacent to the
right end of the area of continuity with the mitral valve, which is
thickened to form the right brous trigone (white dotted area).
Ant.
Sup.
Inf.
Post.
Aorta
Fig. 3.62 The heart is shown in surgical orientation, the parietal
Triangle of Koch
THE VULNERABLE CORONARY

CIRCULATION
On the venous side of the circulation, the
coronary sinus is the only noteworthy
structure related to the valves. The great
walls of the right atrium and ventricle having been removed. The
red line shows the course of the atrioventricular conduction axis as
it penetrates from the atrioventricular node (star) to reach the left
ventricular outow tract. The right bundle branch re-emerges on
the right side beneath the medial papillary muscle.
cardiac vein runs within the left

atrioventricular groove, receiving the
oblique vein as it turns into the inferior part
of the groove (Figures 3.63, 3.64). This
conuence marks the beginning of the
coronary sinus, which proceeds in the fatty
tissue of the inferior atrioventricular

groove to empty into the right atrium
(Figure 3.65). In its course in an adult, it
may approach to within ve to 15
millimetres of the medial attachment of the
mural leaet of the mitral valve. Deeply
Great cardiac vein
87
Base
Ant.
Post.
Apex
Left atrium
Left
ventricle
Fig. 3.63 This cast shows the location of the great cardiac vein in
Oblique vein
Great cardiac vein
Oblique vein
the left atrioventricular groove (white dotted line). The vein does
not become the coronary sinus until it receives the oblique vein of
the left atrium.
Coronary
sinus
Left
Base
Apex
Right
Middle cardiac vein
placed sutures in this area during

replacement of the mitral valve may lead to
damage to the coronary sinus. This can
cause extremely troublesome bleeding. In
addition, when removing a previously
Fig. 3.64 The heart has been removed from the body, and the
diaphragmatic surface is photographed to show the course of the
coronary sinus within the inferior left atrioventricular groove. The
great cardiac vein becomes the coronary sinus at the point where it
receives the oblique vein of the left atrium.
placed mitral valvar prosthesis, care must

be taken not to enter the sinus with
scalpel or suture. The normal anatomy
may well have been distorted by the earlier
operation.
The coronary arteries are intimately

related to both the mitral and tricuspid
valves, as much of their course is within the
atrioventricular grooves. The main stem of
the left coronary artery branches in the
88
Coronary
sinus
Diaphragmatic
surface
of left ventricle
Left
Inf.
Sup.

sternotomy with the surgeon having reected the heart, shows the
position of the coronary sinus in the inferior atrioventricular
groove. The cannula is in the inferior caval vein.
Right
Dominant right coronary artery
Mitral valvar
orifice
Ant.
L
Post.
Circumflex a. stops at obtuse margin
angle of the margin of the left-sided

ventriculoinfundibular fold immediately
above the left brous trigone. The anterior
interventricular artery moves away from
the valves, although its septal perforating
branches extend into the septum
immediately beneath the left-facing leaet
Fig. 3.66 The heart in this image, also shown in Figure 3.41, has a
dominant right coronary artery, which gives rise to the inferior
interventricular artery, and the artery to the atrioventricular node.
As can be seen, the circumex artery is only marginally related to
the mural leaet of the mitral valve in this setting.
of the pulmonary valve. This important

artery could be damaged by extensive
dissection in this area. It is the circumex
branch of the left coronary artery that is
most intimately related to the mitral valve,
particularly when the left coronary artery is
dominant. When the right coronary artery
is dominant, that is, gives rise to the inferior

interventricular artery, as it does in about
90% of cases, the circumex artery (a.) is
related only to the area around the lateral
scallop of the mural leaet of the mitral
valve (Figure 3.66). When the circumex
branch becomes the inferior
interventricular artery, its entire course is

intimately related to the entirety of the
mural leaet (Figure 3.33).
The right coronary artery always runs a
circumferential course around the mural
attachments of the tricuspid valve. The
initial course of the artery is through the
right atrioventricular groove (Figure 3.41),
where it lies on the epicardial aspect of the
ventriculoinfundibular fold. It can be
damaged by deeply placed sutures in this
area21. The artery encircles the attachment
of the mural leaet of the tricuspid valve
before, in the majority of cases, turning to
become the inferior interventricular artery.
Just prior to its descent, the right coronary
artery, when dominant, takes a prominent
U-loop beneath the oor of the coronary
sinus, giving off the artery to the
atrioventricular node at the apex of the
loop. In cases where the circumex artery
gives rise to the inferior interventricular
artery, the atrioventricular nodal artery
originates from the circumex artery
(Figure 3.33). Whether arising from a
dominant left or right coronary artery, this
small but important vessel is related to the
inferior aspect of the annular attachment of
the septal leaet of the tricuspid valve.
References
1. Perloff JK, Roberts WC. The mitral
apparatus. Functional anatomy of mitral
regurgitation. Circulation 1972; 46: 227239.
2. Frater RWM, Anderson RH. How can we
logically describe the components of the
arterial valves? J Heart Valve Dis 2010; 19:
438440.
3. Sievers HH, Hemmer G, Beyersdorf F,

et al. The everyday used nomenclature of
the aortic root components: the Tower of
Babel? Eur J Cardiothorac Surg 2012; 41:
478482.
4. Anderson RH. Demolishing the Tower of
Babel. Eur J Cardiothorac Surg 2012; 41:
483484.
5. Sutton JP 3rd, Ho SY, Anderson RH. The
forgotten interleaet triangles: a review of
the surgical anatomy of the aortic valve.
Ann Thorac Surg 1995; 59: 419427.
6. Angelini A, Ho SY, Anderson RH, Davies
MJ, Becker AE. A histological study of the
atrioventricular junction in hearts with
normal and prolapsed leaets of the mitral
valve. Br Heart J 1988; 59: 712716.
7. Victor S, Nayak VM. The tricuspid valve is
bicuspid. J Heart Valve Dis 1994; 3: 2736.
8. Sutton JP 3rd, Ho Sy, Vogel M, Anderson
RH. Is the morphologically right
atrioventricular valve tricuspid? J Heart
Valve Dis 1995; 4: 571575.
9. Yacoub M. Anatomy of the mitral valve
chordae and cusps. In: Kalmason D (ed).
The Mitral Valve. A Pluridisciplinary
Approach. London: Edward Arnold, 1976;
pp 1520.
10. Kumar N, Kumar M, Duran CM. A
revised terminology for recording surgical
ndings of the mitral valve. J Heart Valve
Dis 1995; 4: 7677.
11. Frater R. Anatomy and physiology of the
normal mitral valve. (Discussion) In:
Kalmanson D (ed). The Mitral Valve. A
Pluridisciplinary Approach. London:
Edward Arnold, 1976; p 41.
12. Lam JHC, Ranganathan N, Wigle ED,
Silver MD. Morphology of the human
mitral valve. I. Chordae tendineae: a
new classication. Circulation 1970; 41:
449458.
89
13. Becker AE, de Wit APM. Mitral valve

apparatus. A spectrum of normality
relevant to mitral valve prolapse. Br Heart J
1979; 42: 680689.
14. Van der Bel-Kahn J, Duren DR, Becker
AE. Isolated mitral valve prolapse: chordal
architecture as an anatomic basis in older
patients. J Am Coll Cardiol 1985; 5: 1335
1340.
15. Roberts WC. The 2 most common
congenital heart diseases. (Editorial) Am J
Cardiol 1984; 53: 1198.
16. Carpentier A, Branchini B, Cour JC, et al.
Congenital malformations of the mitral
valve in children. Pathology and surgical
treatment. J Thorac Cardiovasc Surg 1976;
72: 854866.
17. Victor S, Nayak VM. Denition and
function of commissures, slits and scallops
of the mitral valve: analysis in 100 hearts.
Asia Pacic J Thorac Cardiovasc Surg 1994;
3: 1016.
18. Rosenquist GC, Clark EB, Sweeny LJ,
McAllister HA. The normal spectrum of
mitral and aortic valve discontinuity.
Circulation 1976; 54: 298301.
19. Dodge-Khatami A, Mavroudis C, Backer
CL. Congenital heart surgery nomenclature
and database project: anomalies of the
coronary arteries. Ann Thorac Surg 2000;
69: S270279.
20. Merrick AF, Yacoub MH, Ho SY,
Anderson RH. Anatomy of the muscular
subpulmonary infundibulum with regard to
the Ross procedure. Ann Thorac Surg 2000;
69: 556561.
21. McFadden PM, Culpepper WS
3rd, Ochsner JL. Iatrogenic right
ventricular failure in tetralogy of Fallot
repairs: reappraisal of a distressing
problem. Ann Thorac Surg 1982; 33:
400402.
Surgical anatomy
of the coronary
circulation
Surgical anatomy: coronary circulation
The coronary circulation consists of the

coronary arteries and veins, together with
the lymphatics of the heart. Because the
lymphatics, apart from the thoracic duct,
are of very limited signicance to operative
anatomy, they will not be discussed at any
length in this chapter. The veins, relatively
speaking, are similarly of less interest. In
this chapter, therefore, we concentrate
upon those anatomical aspects of arterial
distribution that are pertinent to the
surgeon, concluding with a brief discussion
of the cardiac venous drainage and the
cardiac lymphatics.
THE CORONARY ARTERIES

The coronary arteries are the rst
branches of the ascending portion of the
aorta. They take their origin from the
sinuses within the aortic root, immediately
above its attachment to the heart
(Figure 4.1). There are three sinuses
within the aortic root, but only two
coronary arteries. The sinuses can be
named, therefore, according to whether
they give rise to an artery, the normal
arrangement being a right coronary, left
coronary, and non-coronary aortic sinus
(Figure 4.2). When described in this

fashion, the terms right and left refer to
the aortic sinuses giving rise to the right
and left coronary arteries, rather than to
the position of the sinuses relative to the
right-to-left coordinates of the body
(Figure 4.3). In the normal heart, the
aortic root is situated obliquely, while in
malformed hearts, the root is frequently
positioned abnormally. Whatever the
position of the aortic root, however, the
two coronary arteries, when two are
present, almost always take origin from
those aortic sinuses that are adjacent to the
sinuses of the pulmonary trunk. Because
of this, it is more convenient, and more
accurate, to consider these sinuses as
being to the left-hand and the right-hand
side of the observer standing, guratively
speaking, within the non-adjacent sinus
and looking towards the pulmonary trunk
(Figure 4.4). This approach to
distinguishing the aortic sinuses giving
rise to the coronary arteries, introduced by
the group from Leiden1, holds true
irrespective of the relationships of the
arterial trunks. It is now conventional to
describe the right-hand sinus as no. 1,
and to distinguish the left-hand sinus as
no. 2. The convention becomes
particularly valuable when considering

coronary arterial origins in malformed
hearts (see Chapter 8).
Irrespective of the specic sinus from
which they arise, the coronary arteries
usually take their origin beneath the
sinutubular junction (Figure 4.5). The
junction is the discrete transition between
the aortic root and the tubular component
of the ascending aorta, and is the most
obvious annular structure within the aortic
root. Deviations of origin of the coronary
arteries relative to the junction are not
uncommon2. They are considered
abnormal, in adults, when arising more
than one centimetre distal to the
sinutubular junction, a feature said to occur
in almost one-twentieth of normal hearts3.
The arterial opening can be deviated either
towards the ventricle, so that the artery
arises deep within the aortic sinus, or
towards the aortic arch, so that the origin is
outside the sinus (Figure 4.6). The
displacement is of greater signicance
when combined with the artery taking an
oblique course through the aortic wall and
originating above or within an
inappropriate aortic sinus. This
arrangement, now known as an anomalous
aortic origin of a coronary artery4, is
Main stem of left

coronary artery
Base
Left
Right
Apex
Aorta
Fig. 4.1 The heart has been dissected by removing the
Removed infundibulum
91
subpulmonary infundibulum. It is photographed in anatomical

orientation to show the origin of the coronary arteries from the
sinuses of the aortic root that lie adjacent to the pulmonary root
(stars). The black dashed line shows the sinutubular junction.
92
Left coronary sinus
Ant.
Base
Apex
Post.
Fig. 4.2 The heart is photographed from above in anatomical
Right coronary sinus
Non-coronary sinus
orientation after removal of the atrial myocardium and the

arterial trunks. Two sinuses of the aortic valve give rise to coronary
arteries, permitting them to be named as the right and left aortic
coronary sinuses. The other sinus is the non-coronary sinus, which
is also non-adjacent relative to the pulmonary trunk.
Left coronary sinus
Left
Post.
Fig. 4.3 The software producing computed tomographic images
Ant.
Right
Non-adjacent sinus
typically associated with the proximal part

of the artery crossing the attachment of the
zone of apposition between adjacent
leaets, and is also considered to represent
one variant of the intramural arrangement
(Figure 4.7). It can involve either the right
also produces markers showing the orientation of structures

relative to the anatomical position. These markers show that the socalled right and left aortic sinuses are not strictly right-sided and
left-sided.
coronary artery arising from the left

coronary aortic valvar sinus (Figures 4.7,
4.8), an arrangement which occurs in one of
every 500 individuals, or the left coronary
artery arising from the right coronary aortic
valvar sinus, found in one of 2000
individuals5. Both variants introduce the

potential for luminal narrowing, and may
provoke disturbances in myocardial
perfusion6.
The left coronary artery almost always
takes origin from a single orice within the
93
Left hand
No.2
Right hand
No.1
Fig. 4.4 The cartoon demonstrates the basis of the so-called
Leiden convention. The surgeon stands, guratively speaking, in

the non-coronary sinus, and looks towards the pulmonary trunk. Of
the sinuses adjacent to the pulmonary trunk, one is thus to the lefthand side. This is sinus no. 2. The other sinus, to the right-hand side,
is sinus no. 1. Almost without exception, the coronary arteries take
origin from one or both of these sinuses. In the usual arrangement,
the right coronary artery arises from sinus no. 1, and the main stem
of the left coronary artery from sinus no. 2.
Tubular aorta
Sup.
Ant.
Post.
Inf.
left-hand facing sinus. In contrast, in about

half of all hearts, there are two orices
within the right coronary aortic sinus
(Figure 4.9). In such instances, the orices
are unequal in size, the larger giving rise to
the main trunk of the right coronary artery,
while the considerably smaller second
orice usually gives rise to an infundibular
artery, or rarely to the artery supplying the
Fig. 4.5 The computed tomogram shows the right and left aortic
sinuses as viewed from behind. It shows the usual situation in
which the coronary arteries arise within the sinuses, proximal to the
sinutubular junction (red dashed line).
sinus node. In one large series7, two orices

were found in the right-hand facing sinus
in almost half the cases, three orices in
7%, and four orices in 2%. In contrast,
multiple orices in the left coronary aortic
sinus (Figure 4.10) are considerably more
rare7. If unrecognised, they may create
problems in the interpretation of coronary
angiograms. The presence of dual orices
in the same sinus, of course, is of far greater

signicance in the setting of an anomalous
aortic origin of a coronary artery, when one
of the orices gives rise to an artery taking
an intra-arterial and intramural course, as
shown in Figures 4.7 and 4.8.
Although rare, the coronary arteries, on
occasion, can arise from a solitary orice in
the aortic root. This is usually within the
94
Sup.
Right
Left
Inf.
Fig. 4.6 In this heart, the aortic valve has been opened and the
aortic root photographed from behind to show the right and left
aortic sinuses. The right coronary artery arises well above the
sinutubular junction.(Reproduced by kind permission of Professor
Anton Becker, University of Amsterdam.)
Intramural course of
right coronary artery
Fig. 4.7 The computed tomogram, in the same orientation as in
Left coronary sinus
right coronary aortic sinus. The artery

originating from the solitary orice can take
one of two patterns. It can divide
immediately into right and left coronary
arteries. The left artery then passes either
in front of the pulmonary trunk
(Figure 4.11), between it and the aorta, or
behind the arterial pedicle, before dividing
Figure 4.5, shows an intramural course of the right coronary artery.

The artery crosses the peripheral attachment of the zone of
apposition between the valvar leaets guarding the right and left
aortic sinuses, and takes its origin, along with the left coronary
artery, from the left aortic sinus (no. 2).
into anterior interventricular and

circumex branches. The solitary artery
can also arise from the left coronary aortic
sinus, branching so that the right coronary
artery passes in front of the pulmonary
trunk (Figure 4.12), runs between the
arterial trunks, or extends behind the
arterial pedicle, running through the
transverse sinus. It is the variants involving

a course between the great arterial trunks
that are of most signicance, as they can be
harbingers of sudden cardiac death6,8.
There are subtle variations in the course
taken by the artery as it passes between the
trunks9. There are three possibilities
(Figure 4.13). In the rst, the artery runs
Fig. 4.8 The tubular aorta has been removed at the level of the
Sup.
Left
Right
Right aortic sinus
Inf.
Orifice of infundibular artery
sinutubular junction to show an anomalous origin of the right

coronary artery from the left aortic valvar sinus, which also gives
rise to the left coronary artery. Note that the right coronary artery
crosses the points of attachment of the valvar leaets to the
sinutubular junction. This is the essence of the intramural
arrangement.
Orifice of right coronary artery
Sup.
Ant.
95
Post.
Fig. 4.9 In this heart, the right coronary artery has a separate
Left aortic sinus
Inf.
between the aorta and the pulmonary trunk

at the level of the aortic sinutubular
junction, as shown in Figures 4.7 and 4.8.
The second possibility is for the artery to
extend deeply within the musculature of
the ventricular septum (Figure 4.14). The
third possibility is for the artery to track
within the tissue plane between the
origin from the right coronary aortic sinus relative to the

infundibular artery.
pulmonary infundibular sleeve and the

aortic root (Figure 4.15). This course is
below the level of the hinges of the
pulmonary valvar leaets, yet not buried
within the muscular ventricular septum.
The fact that the artery is not within the
septum may not readily be appreciated when
seen from the lateral aspect (Figure 4.16). It
is likely that compression on the arterial

lumen will be greatest when the artery is
buried within the musculature of the
septum, an important consideration when
assessing the need for surgical correction of
the anomalous course. A still further
alternative course for a solitary coronary
artery, although less frequent, is when
96
Left coronary sinus
Sup.
Ant.
Post.
Inf.
Fig. 4.10 The computed tomogram shows the separate origin of

Circumflex artery
the interventricular and circumex arteries from the left coronary

aortic sinus.
Left
Sup.
Inf.
Right
Pulmonary trunk
Aorta
the single artery initially follows the path

of the normal right coronary artery. It
then continues beyond the crux,
encircling the left atrioventricular junction
Fig. 4.11 This operative view, seen through a median sternotomy,

shows a solitary coronary artery taking origin from the right
coronary aortic sinus. It divides immediately into right and left
branches, with the main stem of the left coronary artery crossing
the subpulmonary infundibulum. The left coronary artery then
divides into the circumex and anterior interventricular arteries.
through the territory usually supplied by

the circumex artery, before terminating
as the anterior interventricular coronary
artery.
The right and left coronary arteries,

having taken origin from the aortic root,
extend subepicardially within the
atrioventricular and interventricular
97
Main stem of solitary

coronary artery
Sup.
Left
Right
Inf.
Circumflex artery
Ant. intervent. artery
Fig. 4.12 In this heart, there is a solitary coronary artery arising

from the left aortic valvar sinus. It divides into circumex and
anterior interventricular (ant. intervent.) arteries, but also gives rise
to the right coronary artery, which courses across the
subpulmonary infundibulum to reach the right atrioventricular
groove.
Infundibulum
Aortic root
Sup.
Ant.
Fig. 4.13 The long axis section replicating the parasternal

echocardiographic cut shows the tissue plane that exists between
the aortic root and the subpulmonary infundibulum. As shown,
arteries can run between the trunks at the level of the sinutubular
junction (red oval), within the tissue plane between the
subpulmonary infundibulum and the aortic root (blue oval), or
within the substance of the ventricular septum (yellow oval).
Post.
Inf.
grooves, the left-sided artery giving rise to

two major branches. The right coronary
artery emerges from the right aortic sinus
and immediately enters the right
atrioventricular groove (Figure 4.17). It

then encircles the tricuspid orice, running
in the right atrioventricular groove
(Figure 4.18). In approximately
nine-tenths of cases, the right coronary

artery gives rise to an inferior
interventricular artery at the crux, albeit
that the artery is usually said to be
98
Sup.
Left
Right
Inf.
Fig. 4.14 The computed tomogram shows the main stem of the
left coronary artery arising from a solitary coronary artery, which
itself arises from the right coronary aortic sinus. The left coronary
artery extends deeply within the crest of the ventricular septum
(black double-headed arrow) before dividing into its anterior
interventricular and circumex branches.
Sup.
Left
Right
Inf.
Fig. 4.15 This reconstructed computed tomogram shows the

anterior interventricular artery arising from the right coronary
aortic sinus, and extending between the aortic root and the
subpulmonary infundibulum. Unlike the situation shown in
Figure 4.14, however, the artery does not burrow within the crest of
the muscular septum, but runs in the tissue plane separating the
aortic root from the free-standing infundibular musculature.
posterior. Computed tomographic images

now leave no doubt that the artery is
inferior and interventricular (Figure 4.19).
In a good proportion of these cases, the
artery continues beyond the crux, where it
supplies downgoing branches to the

diaphragmatic surface of the left ventricle
(Figure 4.20). This is right coronary
arterial dominance. As the artery encircles
the tricuspid orice, it is most closely
related to the origin of the leaets of the

tricuspid valve near the take-off of its acute
marginal branch. Other important
branches also take origin from this
encircling segment of the artery.
99
Sup.
Ant.
Post.
Inf.
Aortic root
Infundibulum
Fig. 4.16 The computed tomogram shows the course of the

anterior interventricular artery between the infundibulum and the
aortic root previously shown in the reconstruction as Figure 4.15. It
is difcult from the lateral projection to appreciate that the artery
runs within the adipose tissue plane between the infundibulum
and the aortic root, rather than within the musculature of the
ventricular septum (see Figure 4.14).
Left
Apex
Infundibulum
Base
Right
Aorta
Fig. 4.17 The operative view, seen through a median sternotomy,

shows the right coronary artery as it emerges from its sinus into the
right atrioventricular groove.
Immediately after its origin, the artery lies

within the rightward extent of the
transverse sinus, with the adjacent
muscular wall representing the
ventriculoinfundibular fold of the
supraventricular crest. In this course, the
right coronary artery gives rise to
downgoing infundibular branches, which
may also arise by separate orices within
the right aortic sinus. In just over half the
cases, the right coronary artery gives rise

to the artery supplying the sinus node
(Figure 4.21). This artery typically arises
from the proximal part of the right
coronary artery, but on occasion the
nodal artery can arise more distally,
coursing over the lateral margin of the
appendage to reach the terminal groove.
This is of major surgical signicance
(Figure 4.22)10.
The left coronary artery has a short

conuent stem, usually called the left main
artery by the surgeon. It emerges from the
left coronary aortic sinus, and enters the
left margin of the transverse sinus, being
positioned behind the pulmonary trunk
and beneath the left atrial appendage. It is a
very short structure, rarely extending
beyond one centimetre in length before
bifurcating into its anterior interventricular
100
Aorta
Sup.
Right
Left
Fig. 4.18 The computed tomographic image shows the right

coronary artery, having emerged from its aortic valvar sinus,
encircling the tricuspid valvar orice.
Inf.
Right
Post.
Ant.
Left
Fig. 4.19 The computed tomographic image leaves no doubt that
the artery supplying the diaphragmatic surface of the heart is

inferior and interventricular (note the orientation marker in the
lower right corner; F, foot).
and circumex branches (Figure 4.23). In

some hearts, the left main artery trifurcates,
with an intermediate branch present
between the two major branches
(Figure 4.24). The intermediate branch
supplies the pulmonary surface and obtuse
margins of the left ventricle. The anterior

interventricular or descending artery runs
inferiorly within the anterosuperior
interventricular groove, giving off diagonal
branches to the pulmonary surface of the
left ventricle (Figure 4.25), and the
important perforating branches that pass

inferiorly into the septum (Figures 4.26,
4.27). The rst septal perforating branch
(Figure 4.26) is particularly important, as it
is at major risk when the pulmonary valve is
removed for use as a homograft. The
Sup.
101

Right
Left
Inf.
Fig. 4.20 The diaphragmatic surface of the heart has been
Supply to left ventricle
photographed from beneath, with the heart positioned on its apex.

The right coronary artery, having supplied the inferior
interventricular artery, continues to give branches to the
diaphragmatic surface of the left ventricle. This is right coronary
arterial dominance.
Aorta
Superior
caval vein
Left
Sup.
Inf.

sternotomy, shows the artery to the sinus node arising from the
right coronary artery.
Right
interventricular artery then continues

towards the apex, frequently curving under
the apex onto the diaphragmatic surface of
the ventricles.
The circumex branch of the left
coronary artery passes backwards to run in
relation to the mitral orice. Its relationship

to the orice is most extensive when it gives
rise to the inferior interventricular artery at
the crux. In this circumstance, the left
coronary artery is said to be dominant
(Figures 4.28, 4.29). A dominant left
coronary artery, however, is found in only

about one-tenth of cases. When the left
coronary is not dominant, the circumex
artery usually terminates by supplying
branches to the obtuse margin of the left
ventricle. In almost half of normal
102
Surgical incision
Ant.
Fig. 4.22 In this specimen, photographed to replicate the view
Apex
Base
Post.
seen by the surgeon working through a median sternotomy, the

artery to the sinus node takes a lateral origin from the right
coronary artery. It courses over the lateral margin of the right atrial
appendage to reach the terminal groove. It has been divided by
the standard atriotomy.
Sup.
Main stem of left coronary artery
Ant.
Post.
Inf.
Aorta
Circumflex artery
Fig. 4.23 The computed tomographic image shows the left

Obtuse marginal branch
individuals, the circumex artery also

gives rise to the artery that supplies the
sinus node.
Throughout much of their epicardial
course, the arteries and their accompanying
veins are encased in epicardial adipose
coronary artery branching into its anterior interventricular and

circumex branches. Note the diagonal branch (star) arising from
the anterior interventricular artery.
tissue. In some hearts, the myocardium

itself may form a bridge over segments
of the artery (Figure 4.30). The role of
these myocardial bridges in the
development of coronary arterial disease is
not clear. They certainly can be an
impediment to the surgeon in efforts to

isolate the artery.
We have already emphasised the
signicance of the origin of the important
artery supplying the sinus node. This, the
largest of the atrial arteries, originates
103
Left main stem
Circumflex artery
Sup.
Ant.
Inf.
Intermediate artery
Base
Ant.
Post.
Fig. 4.24 The computed tomographic image shows the left

coronary artery giving rise to three, rather than two, branches. The
intermediate branch supplies the obtuse margin of the left
ventricle.
Left main stem

Post.
Apex
Fig. 4.25 This dissection, photographed from the front in

Diagonal branch
from the right coronary artery in just

over half of individuals, and from the
circumex artery in the remainder
(Figure 4.31). There are, however, rare
variants that must also be recognised
when present10. A lateral origin from the
anatomical orientation, shows the course and branches of the

anterior interventricular artery. Note the location of the rst septal
perforating branch (arrow).
right coronary artery, with a course across

the appendage, is an obvious potential
danger for the standard atriotomy
(Figure 4.22). The artery to the sinus node,
rarely, may also take a lateral or terminal
origin from the circumex artery
(Figure 4.31). Although rare in normal

individuals, our experience suggests that
these variants are more frequent in
congenitally malformed hearts11. In
addition, the artery to the sinus node takes
a variable course relative to the cavoatrial
104
Sup.
Base
Apex
Inf.
Aorta
Excised pulmonary root
Fig. 4.26 This close-up view of a specimen, in anatomical

orientation subsequent to excision of the pulmonary valve, shows
the origin of the rst septal perforating artery (arrow). Note its
proximity to the subpulmonary infundibular area, putting it at risk
when the pulmonary valve is removed for use as a homograft.

Left ventricle
Sup.
Ant.
Post.
Inf.
Fig. 4.27 The computed tomographic reconstruction shows the

course of the septal perforating arteries (arrows).
junction. There are three possibilities

(Figure 4.31). Usually, the artery courses
anterocavally across the crest of the
appendage to reach the node. Alternatively,
it runs deeply within Waterstons groove,

and passes retrocavally. It is thus intimately
related to the superior rim of the oval fossa.
The third possibility is for the artery to
branch, forming a circle around the

cavoatrial junction.
The arterial supply to the ventricular
conduction tissues is also of surgical
105
Ant.
Left
Right
Post.
Fig. 4.28 This specimen, dissected and photographed to show the

Artery to AV node
Circumflex artery
base of the heart in anatomical orientation, has a dominant

circumex branch passing behind the mitral orice and giving rise
to the inferior interventricular artery at the crux. AV,
atrioventricular.
Base
Right
Left
Apex
Circumflex artery
Fig. 4.29 The computed tomographic reconstruction shows a

signicance. The atrioventricular nodal

artery arises from the dominant coronary
artery at the crux, usually from a U-turn of
this artery beneath the oor of the coronary
sinus. The nodal artery then passes towards
the central brous body, running within
the brofatty plane forming the meat in
the atrioventricular muscular sandwich.
Having traversed the node in some hearts,
dominant circumex artery, which gives rise to the inferior

interventricular artery.
it perforates the brous atrioventricular

junction to supply a good part of the
branching atrioventricular bundle. The
septal perforating arteries from the anterior
interventricular artery (Figures 4.26, 4.27)
always supply the anterior parts of the
ventricular bundle branches. Occasionally,
they also supply the greater part of the
inferior ventricular conduction tissues.
THE CORONARY VEINS

The coronary veins drain blood from the
myocardium to the right atrium. The
smaller anterior, and the smallest cardiac
veins, drain directly to the cavity of the
atrium. They are not of surgical
signicance. The larger veins accompany
the major arteries, and drain into the
106
Sup.
Right
Left
Inf.
Anterior
interventricular
artery
Fig. 4.30 In this specimen, photographed with the heart

positioned on its apex, there is extensive myocardial bridging across
the anterior interventricular artery (black brace).
Anterocaval course
Origin from right

coronary artery
Origin from
circumflex artery
Fig. 4.31 The cartoon, drawn in anatomical orientation, shows
Distal origin from

circumflex artery
the variations in the origin of the artery to the sinus node, and the
variability relative to the cavoatrial junction. The left-hand panels
show the usual arrangement with the origin from the right
coronary artery, found in 55% of the population, with the rare
variant of a distal origin with coursing across the appendage (lower
left-hand panel). The right-hand panels show a proximal origin
from the circumex artery, found in around 45% of the population,
with the rare variant of a distal origin with coursing across the
dome of the left atrium. The middle panels show the variation
relative to the superior cavoatrial junction. The sinus node is shown
in green.
Retrocaval course
Arterial circle
Distal origin from right
coronary artery
coronary sinus (Figure 4.32). The great

cardiac vein runs alongside the anterior
interventricular artery. It becomes the
coronary sinus as it encircles the mitral
orice to enter the inferior and leftward
margin of the atrioventricular groove. The
coronary sinus then runs within the groove
(Figure 4.33), lying between the left atrial

wall and the ventricular myocardium
(Figure 4.34), before draining into the right
atrium between the sinus septum and the
sub-Eustachian sinus. At the crux, the
sinus receives the middle cardiac vein,
which has ascended with the inferior
interventricular artery, and the small

cardiac vein, which has encircled the
tricuspid orice in company with the
right coronary artery. Occasionally, these
latter two veins drain directly to the
right atrium. The orice of the coronary
sinus is guarded by the Thebesian valve
107
Sup.
Left
Oblique
vein
Right
Inf.
Great
vein
Small vein
Fig. 4.32 The cartoon shows the diaphragmatic surface of the
Middle vein
heart seen from behind, in anatomical orientation. It illustrates the

arrangement of the coronary veins that drain into the coronary
sinus.
Coronary sinus
Coronary
sinus
Diaphragmatic surface
of left ventricle

sternotomy, with the apex of the heart being lifted, shows the
coronary sinus running through the left atrioventricular groove.
(Figure 4.35), which, on very rare

occasions, may be imperforate. A
prominent valve is also found in the great
cardiac vein where it turns around the
obtuse margin of the left ventricle. This is
the valve of Vieussens12. Some consider
that the valve marks the transition from the
great cardiac vein to the coronary sinus. An
alternative view is that the coronary sinus
commences at the site of drainage of the
oblique vein of the left atrium
(Figure 4.36).
THE CARDIAC LYMPHATICS

Little is known about the surgical
implications of the lymphatic drainage of
the heart itself, although lymphatic
structures exist as supercial, myocardial,
and subendocardial networks13. The most
important lymphatic channel within the
thorax, however, is both well recognised
and of particular importance. This is
because, on occasion, the surgeon may
need to ligate this vessel, the thoracic duct,
in patients suffering with problems in

lymphatic drainage subsequent to their
conversion to the Fontan circulation. The
thoracic duct originates within the
abdomen in the conuence of lymphatic
channels known as the cysterna chyli, this
structure lying on the second lumbar
vertebra. It enters the right paravertebral
gutter of the thorax through the aortic
opening of the diaphragm, and runs within
the gutter to the level of the fourth thoracic
vertebra. Within the lower part of the
108
Sup.
Ant.
Post.
Inf.
Great cardiac vein
Mitral valve
Fig. 4.34 In this heart, the musculature of the atrial walls has
Coronary sinus
been removed to show the course of the coronary sinus within the
left atrioventricular groove. It opens into the right atrium at the
base of the triangle of Koch (black double-headed arrow).
Thebesian valve
Left
Sup.
Inf.
Right
Orifice of coronary sinus
Fig. 4.35 This operative view, through a right atriotomy, shows

the Thebesian valve guarding the orice of the coronary sinus.
109
Great cardiac vein
Oblique vein
Coronary sinus
Middle cardiac vein
Sup.
Apex
Fig. 4.36 This preparation was made by casting the coronary sinus
with silastic, the heart then being photographed from behind in
anatomical orientation. The great cardiac vein is seen entering
the coronary sinus. The location of the site of drainage of the
oblique vein marks the point at which the great vein becomes the
coronary sinus.
Base
Inf.
Aorta
Sup.
Ant.
Post.
Thoracic duct
Inf.
thorax, it lies on the vertebral column

between the descending thoracic aorta and
the azygos vein. Crossing the midline
obliquely at the level of the fourth thoracic
vertebra, it enters the left paravertebral
Fig. 4.37 This operative view, taken through a left thoracotomy,

shows the location of the thoracic duct in the vicinity of the left
subclavian artery and descending thoracic aorta.
gutter, running beneath the arch of the

aorta (Figure 4.37). It then continues
superiorly and anteriorly, curving over the
aortic arch between the left common
carotid and subclavian arteries, to
terminate in either the left subclavian or

internal jugular vein as these structures join
to form the left brachiocephalic vein. The
duct has a bromuscular coat, and contains
several valves along its course, with a
110
bifoliate valve characteristically present at

its termination in the brachiocephalic vein.
5.
References cited
1. Gittenberger-de Groot AC, Sauer U,
Oppenheimer-Dekker A, Quaegebeur J.
Coronary arterial anatomy in transposition of
the great arteries: a morphologic study.
Pediatr Cardiol 1983; 4(Suppl 1): 1524.
2. Neufeld HN, Schneeweiss A. Coronary
Artery Disease in Infants and Children.
Philadelphia, PA: Lea & Febiger 1983; pp
7375.
3. Bader G. Beitrag zur Systematic und
Haugkeit der Anomalien der
Coronararterien des Menschen.Virch Arch
Path Anat 1963; 337: 8896.
4. Kaushal S, Backer CL, Popescu AR, et al.
Intramural coronary length correlates with
symptoms in patients with anomalous aortic
6.
7.
8.
9.
origin of the coronary artery. Ann Thorac

Surg 2011; 92: 986992.
Yamanaka O, Hobbs RE. Coronary artery
anomalies in 126,595 patients undergoing
coronary arteriography.Cathet Cardiovasc
Diagn 1990; 21: 2840.
Taylor AJ, Rogan KM, Virmani R. Sudden
cardiac death associated with isolated
congenital coronary artery disease.J Am
Coll Cardiol 1992; 20: 640647.
Engel HJ, Torres C, Page HL Jr. Major
variations in anatomical origin of the
coronary arteries: angiographic
observations in 4,250 patients without
associated congenital heart disease.Cathet
Cardiovasc Diagn 1975; 1: 157169.
Sharbaugh AH, White RS. Single coronary
artery. Analysis of the anatomic variation,
clinical importance, and report of ve cases.
JAMA 1974; 230: 242246.
Torres FS, Nguyen ET, Dennie CJ, et al.
Role of MDCT coronary angiography in
10.
11.
12.
13.
the evaluation of septal vs interarterial

course of anomalous left coronary arteries.
J Cardiovasc Comput Tomogr 2010; 4:
246254.
Busquet J, Fontan F, Anderson RH, Ho
SY, Davies MJ. The surgical signicance of
the atrial branches of the coronary arteries.
Int J Cardiol 1984; 6: 223234.
Barra Rossi M, Ho SY, Anderson RH,
Rossi Filho RI, Lincoln C. Coronary
arteries in complete transposition: the
signicance of the sinus node artery. Ann
Thorac Surg 1986; 42: 573577.
Zawadzki M, Pietrasik A, Pietrasik K,
Marchel M, Ciszek B. Endoscopic study of
the morphology of Vieussens valve.Clin
Anat 2004; 17: 318321.
Walmsley T. The Heart. In: SharpeySchafer E, Symington J, Bryce TH (eds).
Quains Elements of Anatomy. Eleventh
edition Vol IV, Part III. London:
Longmans, Green and Co., 1929; p 110.
Surgical anatomy
of the conduction
system
112
The disposition of the conduction

system in the normal heart has been
emphasised already (see Chapter 2). In
that earlier chapter, we pointed to the
importance, during surgical procedures,
of avoiding the cardiac nodes and
ventricular bundle branches, and
scrupulously protecting the vascular
supply to these structures. In this
chapter, we will consider the anatomy
of these tissues relative to the treatment
of intractable problems of cardiac
rhythm. The abnormal dispositions of
the conduction tissue to be found in
congenitally malformed hearts,
features of obvious signicance to the
congenital cardiac surgeon, will be
discussed in the sections devoted to
those lesions in the chapters that follow.
In this chapter, nonetheless, we
will discuss surgical procedures
performed to treat arrhythmias that
develop in the setting of the Fontan
circulation.
LANDMARKS TO THE
ATRIOVENTRICULAR
CONDUCTION AXIS
In patients with intractable tachycardia, it
may be necessary to ablate the
atrioventricular bundle. Although this

sometimes occurs inadvertently, it can be
surprisingly difcult to divide this
structure intentionally. The landmark to
penetration of the atrioventricular
conduction axis through the brous
insulating plane is the apex of the triangle
of Koch (Figure 5.1). The apex is marked
by the point at which the tendon of Todaro
inserts into the central brous body
(Figure 5.2). Just inferior to the apex of this
triangle, the components of the
atrioventricular node gather themselves
together, and enter the insulating tissues of
the brous body (Figures 5.3, 5.4). Once
insulated from the atrial myocardial mass,
the conduction axis becomes the
penetrating atrioventricular bundle, better
known as the bundle of His. This part of
the overall atrioventricular conduction axis
is short, but extends leftwards as it pierces
the brous body. As the axis passes
through the brous tissue, and divides into
its branches, the brous tissue, which
forms the membranous septum, is crossed
by the septal leaet of the tricuspid valve,
dividing it into atrioventricular and
interventricular components. The
interventricular component of the septum
is often occupied by the non-branching
component of the conduction axis. Further
Septal leaflet of tricuspid valve
anteriorly, the axis reaches the crest of the

muscular septum, immediately beneath the
brous membranous septum (Figure 5.5).
It divides here, with the branches of the left
bundle cascading down the smooth left
surface of the muscular ventricular septum.
When viewed from the left, the branching
bundle is intimately related to the subaortic
outow tract, with the brous triangle
separating the non-coronary and right
coronary leaets of the aortic valve marking
the take-off of the left bundle branches
(Figure 5.6). Sometimes, the branching
bundle lies below the septal crest, being
carried on the left ventricular aspect of the
septum1. Having given rise to the left
bundle branches, the right bundle branch
takes origin from the axis, burrowing
intramyocardially to reach the right side of
the septum, and surfacing beneath the
medial papillary muscle. The axis itself
continues beyond the origin of the right
bundle branch, entering the aortic root as
the dead-end tract2. Often unrecognised,
this rudimentary structure may prove to be
signicant as a substrate for outow tract
tachycardias. Taken together, the site of the
conduction axis can be determined by
taking a line from the apex of the triangle of
Koch to the medial papillary muscle
(Figure 5.7). In terms of the atrial
Left
Sup.
Inf.
Right
Fig. 5.1 The operative photograph shows the surgeons view
Tendon of Todaro
Coronary sinus
through a right atriotomy in a patient with an atrial septal defect in

the oval fossa (star). The landmarks of the triangle of Koch (white
dashed lines; red circle marks its apex) are obvious. Tension has
been placed on the Eustachian valve to bring the tendon of Todaro
into prominence.
Surgical anatomy: conduction system
113
Septal leaflet
Left
Inf.
Sup.
Fig. 5.2 Another operative view, in the same orientation as in
Tendon of Todaro
Right
Figure 5.1, shows the tendon of Todaro inserting into the

atrioventricular part of the membranous septum.
Atrial
septum
Mitral
valve
Ventricular
septum
Bundle of His
Tricuspid
valve

Tendon
of Todaro
Compact node
components of the axis, the compact

atrioventricular node, located within the
triangle of Koch, is positioned some
distance posterior to the attachment of the
septal leaet of the tricuspid valve. It is well
superior to the orice of the coronary sinus.
VENTRICULAR PRE-EXCITATION
Ventricular pre-excitation is a frequent
problem of cardiac rhythm that necessitates
knowledge of the pertinent anatomy for its
optimal treatment. The arrhythmia occurs
orientation, shows the location of the

conduction tissues within the triangle of Koch,
and the mechanism of penetration of the axis
of conduction tissue into the central brous
body to form the bundle of His, the point of
penetration serving to delimit the junction of
the atrioventricular node with the penetrating
bundle.
when all, or part, of the ventricular

myocardium is excited earlier than would
be expected had the impulse reached the
ventricles by way of the normal
atrioventricular conduction system3. There
are various anatomical pathways, proven
114
Atrial myocardium
Penetrating atrioventricular bundle

Left
Base
Apex
Fig. 5.4 The histological section shows the location of the

Right
penetrating atrioventricular bundle as an insulated structure

within the central brous body. The section corresponds to the
upper right-hand panel of Figure 5.3.
Left bundle branch
Left
Base
Apex
Right
Ventricular septum
Fig. 5.5 The histological section, in the same anatomical
Septal leaflet of
tricuspid valve
Branching atrioventricular bundle
and hypothetical, that can produce this

phenomenon. Essentially, they are
pathways that short circuit part, or all, of
the normal delay induced within the
atrioventricular conduction axis. Most of
this delay occurs within the atrioventricular
node and its zones of transitional cells, but
an increment of delay reects the time
orientation as in Figure 5.4, shows the branching component of the

atrioventricular conduction axis. It lies astride the crest of the
muscular ventricular septum, sandwiched between the septum and
the central brous body. (Courtesy of Professor Anton Becker,
University of Amsterdam.)
taken for the impulse to traverse the

ventricular bundle branches, as these
structures are insulated from the septal
myocardium. Accessory pathways can exist
between the atrium and the atrioventricular
bundle, producing atrio-Hisian tracts,
and between the conduction axis and the
crest of the ventricular septum, the latter
arrangement rst being described by

Mahaim as paraspecic connections4.
These are not amenable to surgical
division. In contrast, the accessory
atrioventricular pathways that produce the
WolffParkinsonWhite syndrome,
probably the most common form of
pre-excitation, are very much amenable to
115
Non-coronary aortic leaflet
Right coronary aortic leaflet
Left bundle branch
Membranous septum
Fig. 5.6 The diagram is a reproduction of the original drawing

made by Tawara. It shows the location of the left bundle branch (in
red) relative to the aortic root.
Ant.
Sup.
Inf.
Post.
Aorta
Fig. 5.7 The dissection was made by removing the parietal walls
Triangle of Koch
surgical division5. Now, however, if

division is necessary, the pathways will
almost certainly be ablated in the catheter
laboratory. Surgical treatment of this
of the right atrium and ventricle. It is viewed in surgical orientation,

showing the location of the atrioventricular conduction axis (red
line). The star shows the position of the atrioventricular node.
arrhythmia, nonetheless, constituted an

important step in the evolution of cardiac
surgery. When treatment was rst mooted,
the bundles were called, inappropriately,
bundles of Kent5. The atrioventricular

muscular strands that are part of the circuit
responsible for the abnormal rhythm join
together the atrial and ventricular
116
myocardial muscle masses outside the area

of the specialised conduction tissues
(Figure 5.8). The best initial description of
the bundles was given by Ohnell6. His
illustration (Figure 5.9) shows that the
structures, which typically extend through
the fat pad on the epicardial aspect of the
valvar annulus, bear no resemblance to the
node-like remnants described by Kent, and

inappropriately considered by him to be
part of the normal conduction system
(Figure 5.10). In reality, they are
remnants of a ring of specialised
conducting tissue that surrounds the
atrioventricular canal of the developing
heart. They are found as complete rings in
animals such as the mouse or rat7, and the

remnants can be found by careful
examination of human hearts8. Only in
abnormal situations, however (see later), do
the node-like remnants give rise to
anomalous muscular atrioventricular
connections. The abnormal accessory
muscular bundles that are the substrate for
Accessory atrioventricular connection
Atrial wall
Ventricular wall
Base
Right
Left
Apex
Annulus
Fig. 5.8 The histological section across the atrioventricular

junction shows the arrangement of a typical left-sided accessory
muscular atrioventricular connection. (Courtesy of Professor Anton
Becker, University of Amsterdam.)
Fig. 5.9 The original drawing made by Ohnell shows the

arrangement of the typical left-sided accessory muscular
atrioventricular connections. Comparison with Figure 5.8 shows the
accuracy achieved by Ohnell when making his drawing.
117
Atrium
Node
Ventricle
Valve
the WolffParkinsonWhite syndrome

(Figure 5.8) can be found anywhere around
the atrioventricular junctions. They are
best described as being left-sided, rightsided, and paraseptal. The anatomy of each
group shows signicant differences.
Left-sided pathways are found at any
point around the mural component of the
mitral valvar orice. Pathways can extend
through the area of aortic-mitral valvar
brous continuity, but are exceedingly
rare. The pathways almost always cross
from the atrial to the ventricular muscle
masses outside a well-formed brous
annulus, with the brous structure
supporting the hinge of the mural leaet of
the mitral valve (Figure 5.8). The atrial
origins of the connections are very close to
the brous junction9. The bundles
themselves usually skirt very close to the
brous tissue, often branching into several
roots, which then insert into the ventricular
myocardium. The bundles are rarely
thicker than one to two millimetres in
diameter, and are made up of ordinary
working cardiomyocytes. On occasions,
there may be more than one bundle in the
Fig. 5.10 The illustration is taken from one of Kents

demonstrations to the Society of Physiology, and relabelled by us. It
shows the structure of remnants of conduction tissue found
adjacent to the right atrioventricular junction. The node-like
structure bears no resemblance to the accessory connections that
produce WolffParkinsonWhite syndrome (see Figures 5.8 and 5.9),
but can rarely give rise to an anomalous connection (see
Figure 5.16).
same patient9. If approached from within

the atrium, incisions that divide the atrial
myocardium, or ablative lesions placed
above the origin of the leaets of the mitral
valve, are unlikely to divide the accessory
muscle bundles themselves. In order to
ablate the accessory connection surgically,
it was usually necessary to dissect within
the fat pad on the epicardial aspect of the
annulus, or to approach the pathway from
the epicardium. If approached
endocardially, it was necessary to reect the
coronary vessels to expose the accessory
muscle bundles. When treated in the
catheter laboratory, lesions are usually
placed on the ventricular aspect of the
hingepoint of the mitral valve.
Right-sided accessory pathways may
also pass through the fat pad to connect
the atrial and ventricular myocardial
masses. More frequently, bundles on the
right side are found some distance away
from the attachment of the leaets of the
tricuspid valve, which is rarely a rm and
well-formed brous junction, as is usually
the case on the left side. Right-sided
connections can be multiple, and can
coexist with left-sided pathways. They are

frequently associated with Ebsteins
malformation, and may need to be treated
concomitantly with repair or replacement
of the abnormal tricuspid valve. They can
be found at any point within the parietal
aspect of the tricuspid orice, from the
site of the membranous septum to the
mouth of the coronary sinus
(Figure 5.11). The same rules for their
ablation apply as discussed for left-sided
connections.
It is connections in the paraseptal
position that constitute the greatest
clinical challenge10. When viewed from
the right atrium, they can cross from the
atrial to the ventricular myocardium
(Figure 5.12) at any point between the
mouth of the coronary sinus and the
supraventricular crest. They present
problems for ablation, rstly, because
they may run deep within the oor of the
triangle of Koch as viewed from the right
atrium. The second problem is that the
atrioventricular node and bundle are also
found within this area. The anatomy of
the area is best illustrated by dissections
118
Left
Ant.
Post.
Right
Aorta
Tricuspid
valvar
orifice
Fig. 5.11 The dissection, photographed in anatomical orientation

from above and behind, shows the potential sites of
atrioventricular connections (black dashed line) in the parietal
aspect of the right atrioventricular junction.
Potential sites of right-sided

atrioventricular connections
Left
Apex
Base
Ventricular septum
Right
Atrial myocardium
Fig. 5.12 The histological section, in anatomical orientation,
Accessory AV connection
shows a muscular accessory atrioventricular (AV) connection

crossing the brofatty insulating plane of the right atrioventricular
junction. The strand takes its origin from the distal insertion of the
atrial myocardium into the area of attachment of the septal leaet
of the tricuspid valve, and crosses the groove to attach to the
ventricular myocardium. (Courtesy of Professor Anton Becker,
University of Amsterdam.)
Lumen
that have removed the atrial walls from

the base of the ventricular mass
(Figure 5.13). Such dissections reveal the
cranial continuation of the inferior
atrioventricular groove, which extends as
a layer of broadipose tissue between the

atrial and ventricular muscle masses,
reaching the central brous body when
traced cranially. The artery to the
atrioventricular node courses forwards
within this tissue plane (Figure 5.13). The

atrioventricular node itself occupies the
superior part of the atrial layer of this
triangular sandwich, with the
broadipose tissue representing the
119
Artery to AV node
Membranous septum
Right atrium
Left atrium
Fig. 5.13 The dissection, shown in anatomical orientation,
Circumflex coronary artery
meat between the muscular layers of the

sandwich. Accessory muscular
connections may cross through this
insulating layer at any point from the
attachment of the mitral and tricuspid
valves at either side of the muscular
ventricular septum. Indeed, the only
connection that has been identied
morphologically within this area was
located at the insertion of the tricuspid
valve (Figure 5.12)9. If necessary, the
broadipose tissue plane can be entered
surgically from the cavity of the right
atrium, or can be reached by dissection
from the epicardial aspect. Incisions
within the atrial component of this
atrioventricular sandwich, interrupting
the muscular approaches to the
atrioventricular node, have also been
shown to interrupt reciprocating
atrioventricular nodal tachycardias11.
Treatment of these latter arrhythmias is
also accomplished now with efciency and
safety using catheter ablation
(Figure 5.14). When treating these
arrhythmias, either surgically or by
illustrates the plane occupied by adipose tissue that runs superiorly

and anteriorly beneath the mouth of the coronary sinus. The right
and left atrial walls have been resected (blue and yellow dotted
lines). The star shows the apidose tissue occupying the inferior
atrioventricular groove. The artery to the atrioventricular (AV)
node runs within the adipose tissue, in this case from a dominant
left coronary artery.
catheter ablation, it should be

remembered that the triangle of Koch
contains both the atrioventricular node
and its nutrient artery. Unless performed
with care, there is always the danger that
intervention can produce complete
atrioventricular dissociation.
Surgeons and catheter ablationists, when
attempting to treat arrhythmias, have
conventionally referred to the oor of the
triangle of Koch as being septal. They also
describe an anterior septum in the region
cranial to the membranous septum12. By
this, they mean the area of the right
atrioventricular junction that lies adjacent to
the supraventricular crest. It is a mistake to
describe this part of the right
atrioventricular junction as being septal, just
as it is incorrect to consider the atrial aspect
of the triangle of Koch as representing a
septal structure. In reality, the so-called
anterior septum is the medial margin of the
ventriculoinfundibular fold. If muscular
atrioventricular connections exist in this
area, they will join the atrial wall to the
supraventricular crest of the right ventricle
(Figure 5.15). Experience has also shown

that muscular connections running to the
most lateral margin of the supraventricular
crest at the acute margin of the right
ventricle can produce the
electrocardiographic pattern initially
attributed to the so-called Mahaim
connections13. Such connections, when
removed surgically from the acute margin,
were shown to resemble the tissues of the
atrioventricular node histologically. We had
previously identied such a pathway
running across the right atrioventricular
junction (Figure 5.16)9. The atrial
component of these connections is
remarkably reminiscent of the illustrations
provided by Kent (Figure 5.10). Kent had
argued, incorrectly, that the nodal remnants
were pathways for normal atrioventricular
conduction14. Under abnormal
circumstances, nonetheless, these node-like
remnants are able to function as the atrial
origin of specialised muscular accessory
connections15. It is these connections that
now are known to produce ventricular preexcitation of the Mahaim type.
120
Triangle of Koch
Oval fossa
Sup.
Fig. 5.14 The photograph shows the site of ablation at the base of
Post.
Ant.
Site of ablation
Inf.
the triangle of Koch, which cured a patient with atrioventricular nodal

re-entry tachycardia. It is outside the area occupied by the specialised
tissues of the atrioventricular node (star) and its zones of transitional
cells.(Reproduced by kind permission of Dr Wyn Davies, St Marys
Hospital, London.)
Non-coronary leaflet of aortic valve
Sup.
Left
Right
Inf.
Fig. 5.15 The dissection, in anatomical orientation, illustrates

SUBSTRATES FOR OTHER

SUPRAVENTRICULAR
TACHYCARDIAS
The attention of both surgeons and
interventionists has focused on the
how the area anterior to the membranous septum, which was

previously considered to represent the anterior septum, is part of
the parietal wall of the right atrioventricular junction.
substrates of other supraventricular

tachycardias, particularly atrial utter and
brillation. Atrial utter of the commonest
type has shown itself to be especially
amenable to interventional therapy. The
usual utter circuit is known to pass in
counter-clockwise fashion (Figure 5.17),

running down the terminal crest before
ascending through the septal isthmus of the
tricuspid valvar vestibule16. The most
inferior part of the circuit passes through
another muscular isthmus, this time
Accessory AV connection
Node of Kent
Left
Apex
Base
121
Right
Terminal crest
Fig. 5.16 The section is taken across the right atrioventricular (AV)
junction, and comes from the heart of a patient who had ventricular
pre-excitation. The tricuspid valve was deformed by Ebsteins
malformation. An accessory muscular connection was identied
taking origin from a nodal remnant as identied by Kent, and
crossing the insulating plane to run within the muscularised leaet
of the tricuspid valve.(Section reproduced by kind permission of
Professor Anton Becker, University of Amsterdam.)
Sup.
Left
Right
Inf.
Septal isthmus
Cavotricuspid isthmus
limited by the orice of the inferior caval

vein and the hinge of the septal leaet of the
tricuspid valve (Figure 5.18). The isthmus
has three discrete areas, containing various
combinations of brous and muscular
tissue (Figure 5.19)17. Interventionists now
Fig. 5.17 The right atrium has been opened and photographed in
anatomical orientation to show the circuit (arrows) known to be
responsible for the common variant of atrial utter. The utter
wave descends the terminal crest, crosses through the inferior
cavotricuspid isthmus, and then ascends through the septal isthmus
to reach the superior aspect of the terminal crest before
recommencing the circuit.
are able to construct lines of block with

great facility so as to divide the circuit. Care
should be taken in the vestibular area to
avoid damage to the right coronary artery
(Figure 5.19). Such lines obviously would
be made surgically with equal facility. This
is the reasoning behind the surgical

manoeuvres performed as part of the
treatment of patients with the Fontan
circulation, known as the Fontan
conversion18, to be discussed in greater
detail later.
122
Coronary sinus
Sup.
Ant.
Post.
Inf.
Oval fossa
Tricuspid valve
Posterior
Fig. 5.18 The right atrium has been opened through the parietal
wall, and photographed in anatomical orientation to show the
structure of the inferior cavotricuspid isthmus. It has posterior,
middle, and anterior components (black braces).
Anterior
Middle
Posterior-Fibrous
Anterior-Smooth
Lumen of right atrium
Fig. 5.19 This histological section,
Inf. caval vein

Middle-Pectinated
The other arrhythmia now becoming

increasingly amenable to interventional
therapy is atrial brillation. It has long been
known that surgical techniques that create
mazes19 or corridors20 can certainly
ameliorate, if not cure, this troublesome
entity. The operative procedures are
complex and time consuming. The maze
procedure, for example, has undergone
many modications21, details of which are
beyond the context of our description,
although we describe the third iteration
Tricuspid valve
designed by Cox when considering

treatment of postoperative arrhythmias
(see later). More recently, interventionists
have shown that it is feasible to construct
lines of block within both the left and right
atriums, lesions which can provide
successful treatment for atrial
brillation22,23. It has also been shown that
a proportion of cases of brillation can be
cured by making focal lesions in the mouth
of the pulmonary veins24. This is because
the atrial myocardium extends for variable
prepared by Professor Siew Yen Ho, shows

the structure of the three components of the
inferior cavotricuspid isthmus. Note the
adjacency of the right coronary artery to the
vestibular musculature in the anterior
compartment.
distances along the pulmonary veins from

the venoatrial junctions (Figure 5.20).
These sleeves of myocardium contain
bundles of cardiomyocytes running in
different directions, along with separating
sheaths of brous tissue, which set the
scene for the focal triggering that produces
the brillation25,26. It has been suggested
that the cardiomyocytes within the sleeves
are histologically specialised27. This is a
spurious claim. The cardiomyocytes are all
of working myocardial origin, with the
123
Dome of left atrium
Fig. 5.20 The dissection, made by Professor Damien SanchezQuintana, shows the dome of the left atrium, the epicardium
having been removed to illustrate the organisation of the
myocardial bres. Sleeves of myocardium (arrows) can be seen to
extend onto the pulmonary veins for varying distances. These
sleeves are now known to be the sources of focal activity in some
variants of atrial brillation.
Fig. 5.21 The pulmonary root has been opened, and the valvar
Semilunar hinges of
pulmonary valvar
leaflets
Proximal attachments to infundibulum
pulmonary venous myocardium never

having had the characteristics of
conduction tissues28.
SUBSTRATES FOR OUTFLOW

TRACT TACHYCARDIAS
Experience shows that some ventricular
tachycardias can be cured by placing
lesions in the region of the bases of the
arterial valvar sinuses29. Such lesions are
placed most frequently in the adjacent
sinuses of the aorta and pulmonary trunk.
As we have shown in Chapter 3, these
sinuses, at their bases, contain crescents of
leaets removed, to show the muscular crescents (stars)

incorporated at the base of the three pulmonary valvar sinuses.
These muscular crescents can be the site of substrates for outow
tract tachycardias.
ventricular muscle, as the semilunar hinges

of the valvar leaets cross the anatomical
ventriculoarterial junctions (Figures 5.21,
5.22). It is most likely that the abnormal
rhythms producing the outow tract
tachycardias take their origin within these
myocardial crescents. It is also known,
however, that abnormal rhythms can rarely
be cured by ablative lesions placed in the
non-adjacent sinus of the aortic root29. It is
rare to nd myocardium within the base of
this non-adjacent sinus. Sometimes the
aortic valve, like the pulmonary valve, can
be supported by a complete muscular
infundibulum30. If present, such
infundibular musculature would produce a
crescent within the non-adjacent sinus. In

the absence of such musculature, however,
it must be presumed that the abnormal
rhythm has an extraventricular origin. It is
also known that, sometimes, the outow
tract tachycardias are ablated by lesions
placed distal to the ventriculoarterial
junction31. During their development, the
arterial roots are encased within turrets of
outow tract myocardium, which then
regress as part of the normal developmental
process32. Persistence of parts of this sleeve
of outow tract myocardium provides the
best explanation for the origins of those
arrhythmias originating distal to the
124

Continuity with mitral valve
Proximal muscular attachments
Fig. 5.22 The aortic root has been opened, and the valvar leaets
removed. There is muscle at the base of the two aortic valvar sinuses
giving rise to the coronary arteries (stars), but not in the nonadjacent sinus.
Amputated
right appendage
Tricuspid valve
Coronary sinus
Oval fossa
Fig. 5.23 The cartoon shows the lesions made within the right
Superior caval vein
Inferior caval vein
ARRHYTHMIA SURGERY IN
PATIENTS WITH CONGENITAL
HEART DISEASE
As patients get older, subsequent to surgical
correction of congenital cardiac lesions,
increasing numbers present with arrhythmic
problems33. The enhanced understanding of
the macro-re-entrant circuits responsible for
the abnormal rhythms has led to
modications of surgical techniques.
Advances in the design of pacemakers and
atrium during the Fontan conversion procedure so as to avoid

postoperative problems with rhythm.
implantable cardiac debrillators have also

contributed to improved treatments33, but
such details are beyond the scope of our
current discussion. Knowledge of the
anatomical background for the created
therapeutic lines of block, nonetheless, now
achieves increasing signicance. The
commonest arrhythmias requiring treatment
are macro-re-entrant atrial tachycardia, atrial
brillation, and ventricular tachycardia.
Such problems are themselves encountered
most frequently in postoperative patients
with functionally univentricular hearts, and

those who have undergone surgical
treatment of tetralogy of Fallot or
transposition. The reasoning behind the
therapeutic approach is to transform areas of
slow conduction to areas of no conduction34.
This is achieved by interrupting myocardial
corridors or isthmuses between obstacles or
scars, while preserving sinus rhythm and
normal atrioventricular conduction.
The commonest operations are the
right atrial maze, typically performed for
Atrial septal defect
125
Right
Sup.
Inf.
Left
Cryothermy lesion
Fig. 5.24 The cryoprobe is photographed in position to produce a

lesion from the atrial septal defect to the coronary sinus during a
Fontan conversion procedure, in a patient with tricuspid atresia.
Aorta
Tricuspid atresia
Coronary sinus
Cryocath probe
Pulmonary trunk
Fig. 5.25 The cartoon shows the lesions producing the right
Cannula in superior
caval vein
Cannula in inferior
caval vein
those with macro-re-entrant tachycardia,

and the Cox maze III procedure for
patients with atrial brillation. The right
atrial maze procedure involves the
resection of the tip of the right atrial
appendage, and part of its anterior surface
if the appendage is signicantly enlarged,
as is often the case in patients with failed
Fontan circulations. Lines of block are
atrial maze procedure as part of the Fontan conversion

procedure.
then created from the edge of the resected

appendage to the oval fossa, from the
oval fossa across the terminal crest, and
then a series of lines to divide the
cavotricuspid isthmus (Figure 5.23). The
lines dividing the isthmus run from the
edge of the oval fossa to the margin of the
coronary sinus (Figure 5.24), from the
mouth of the coronary sinus to the mouth
of the inferior caval vein, and from the

inferior caval vein to the hinge of the
septal leaet of the tricuspid valve. The
last lesion is obviously not possible in
those with tricuspid atresia due to
absence of the right atrioventricular
connection, but the other lines can
easily be created in this setting
(Figure 5.25).
126
Aorta
Amputated left
appendage
Tricuspid valve
Mitral valve
Pulmonary trunk
Fig. 5.26 The cartoon shows the lesions produced in the third
Cannula in superior
caval vein
Cannula in inferior
caval vein
In the third iteration of the series of

procedures designed by Cox for treatment
of patients with atrial brillation
(Figure 5.26)21, the lines of block can be
produced either by surgical incisions, or
by cryoablation. An extensive line is
created within the roof of the left
atrium, encircling the orices of all
pulmonary veins. A line of block is
then created to the mouth of the left
atrial appendage, which can be excised,
or alternatively encircled by another line.
A linear lesion is then made from the line
encircling the pulmonary veins to the
annulus of the mitral valve in the region of
the third scallop of the mural leaet. The
nal lesion is placed epicardially across
the coronary sinus as it runs within the
left atrioventricular groove. If using
cryoablation, the nal lesion is maintained
for two minutes, while the other requires
only one minute of freezing at minus
160 degrees Celsius.
References cited
1. Massing GK, James TN. Anatomical
conguration of the His bundle and bundle
branches in the human heart. Circulation
1976; 53: 609621.
iteration of the Cox maze procedure for treatment of atrial

brillation.
2. Kurosawa H, Becker AE. Dead-end tract of

the conduction axis. Int J Cardiol 1985; 7:
1320.
3. Durrer D, Schuilenburg RM, Wellens HJJ.
Pre-excitation revisited. Am J Cardiol 1970;
25: 690698.
4. Mahaim I. Maladies Organiques du Faisceau
de His-Tawara. Paris: Masson et Cie., 1931.
5. Sealy WC, Gallagher JJ, Pritchett ELC. The
surgical anatomy of Kent bundles based on
electrophysiological mapping and surgical
exploration. J Thorac Cardiovasc Surg 1978;
76: 804815.
6. Ohnell RF. Preexcitation, a cardiac
abnormality. Pathophysiological,
pathoanatomical and clinical studies of an
excitatory spread phenomenon. Acta Med
Scand 1944; 152: 14167.
7. Yanni J, Boyett MR, Anderson RH,
Dobrzynski H. The extent of the
specialized atrioventricular ring tissues.
Heart Rhythm 2009; 6: 672680.
8. Anderson RH, Davies MJ, Becker AE.
Atrioventricular ring specialized tissue in
the normal heart. Eur J Cardiol 1974; 2:
219230.
9. Becker AE, Anderson RH, Durrer D,
Wellens HJJ. The anatomical substrates
of WolffParkinsonWhite syndrome.
A clinicopathologic correlation in
seven patients. Circulation 1978; 57:
870879.
10. Sealy WC, Gallagher JJ. The surgical

approach to the septal area of the heart
based on experience with 45 patients with
Kent bundles. J Thorac Cardiovasc Surg
1980; 79: 542551.
11. Johnson DC, Ross DL, Uther JB. The
surgical cure of atrioventricular junctional
reentrant tachycardia. In: Zipes DP, Jalife J
(eds). Cardiac Electrophysiology from Cell to
Bedside. London: W.B. Saunders, 1990; pp
921923.
12. Guiraudon GM, Klein GJ, Sharma AD,
et al. Surgical approach to anterior septal
accessory pathways in 20 patients with the
WolffParkinsonWhite syndrome. Eur J
Cardiothorac Surg 1988; 2: 201206.
13. Guiraudon CM, Guiraudon GM, Klein GJ.
Nodal ventricular Mahaim pathway:
histologic evidence for an accessory
atrioventricular pathway with an AV
node-like morphology. Circulation 1988; 78
(Suppl 2): 40.
14. Kent AFS. The structure of the cardiac
tissues at the auriculo-ventricular junction.
J Physiol 1913; 47: 1718.
15. Anderson RH, Ho SY, Gillette PC, Becker
AE. Mahaim, Kent and abnormal
atrioventricular conduction. Cardiovasc Res
1996; 31: 480491.
16. Cosio FG, Lopez-Gil M, Giocolea A,
Arribas F, Barroso JL. Radiofrequency
ablation of the inferior vena cavatricuspid
17.
18.
19.
20.
21.
22.
valve isthmus in common atrial utter. Am

J Cardiol 1993; 71: 705709.
Cabrera JA, Sanchez-Quintana D, Ho SY,
Medina A, Anderson RH. The architecture
of the atrial musculature between the
orice of the inferior caval vein and the
tricuspid valve: the anatomy of the isthmus.
11861195.
Mavroudis C, Backer CL, Deal BJ,
Johnsrude C, Strasburger J. Total
cavopulmonary conversion and maze
procedure for patients with failure of the
Fontan operation. J Thorac Cardiovasc Surg
2001; 122: 863871.
Cox JL, Boineau JP, Schuessler RB,
Jaquiss RD, Lappas DG. Modication of the
maze procedure for atrial utter and atrial
brillation. I. Rationale and surgical results.
J Thorac Cardiovasc Surg 1995; 110:
473484.
Defauw JJ, Guiraudon GM, van Hemel NM,
et al. Surgical therapy of paraoxysmal atrial
brillation with the corridor operation. Ann
Thorac Surg 1992; 53: 564570.
Cox JL, Ad N. New surgical and catheterbased modications of the Maze procedure.
Semin Thorac Cardiovasc Surg 2000; 12:
6873.
Goya M, Ouyang F, Ernst S, et al.
Electroanatomic mapping and catheter
ablation of breakthroughs from the right
23.
24.
25.
26.
27.
28.
atrium to the superior vena cava in patients

with atrial brillation. Circulation 2002; 106:
13171320.
Pappone C, Oreto G, Rosanio S, et al. Atrial
electroanatomic remodelling after
circumferential radiofrequency pulmonary
vein ablation: efcacy of an anatomic
approach in a large cohort of patients with
atrial brillation. Circulation 2001; 104:
25392544.
Shah DC, Haissaguerre M, Jais P. Catheter
ablation of pulmonary vein foci for atrial
brillation. PV foci ablation for atrial
brillation. Thorac Cardiovasc Surg 1999;
47(Suppl 3): 352356.
Ho SY, Cabrera JA, Tran VH, et al.
Architecture of the pulmonary veins:
relevance to radiofrequency ablation. Heart
2001; 86: 265270.
Hocini M, Ho SY, Kawara T, et al.
Electrical conduction in canine pulmonary
veins. Electrophysiological and anatomical
correlation. Circulation 2002; 105:
24422448.
Perez-Lugones A, McMahan JT, Ratliff
NB, et al. Evidence of specialized
conduction cells in human pulmonary veins
of patients with atrial brillation. J
Cardiovasc Electrophysiol 2003; 14:
803809.
Mommersteeg MT, Christoffels VM,
Anderson RH, Moorman AF. Atrial
29.
30.
31.
32.
33.
34.
127
brillation: a developmental point of view.

Heart Rhythm 2009; 6: 18181824.
Asirvatham SJ. Correlative anatomy for
the invasive electrophysiologist:
outow tract and supravalvar arrhythmia.
955968.
Rosenquist GC, Clark EB, Sweeney LJ,
McAllister HA. The normal spectrum of
mitral and aortic valve discontinuity.
Circulation 1976; 54: 298301.
Timmermans C, Rodriguez LM, Crijns HJ,
Moorman AF, Wellens HJ. Idiopathic left
bundle-branch block-shaped ventricular
tachycardia may originate above the
pulmonary valve. Circulation 2003; 108:
19601967.
Sizarov A, Lamers WH, Mohun TJ,
et al. Three-dimensional and molecular
analysis of the arterial pole of the
developing human heart. J Anat 2012; 220:
336349.
Karamlou T, Silber I, Lao R, et al.
Outcomes after late reoperation in
patients with repaired tetralogy of Fallot:
the impact of arrhythmia and arrhythmia
surgery. Ann Thorac Surg 2006; 81:
17861793.
Mavroudis C, Deal BJ, Backer CL, Tsao S.
Arrhythmia surgery in patients with and
without congenital heart disease. Ann
Thorac Surg 2008; 86: 857868.
Analytical description
of congenitally
malformed hearts
Congenitally malformed hearts
Systems for describing congenital cardiac

malformations have frequently been based
upon embryological concepts and theories.
As useful as these systems have been, they
have often had the effect of confusing the
clinician, rather than clarifying the basic
anatomy of a given lesion. As far as the
surgeon is concerned, the essence of a
particular malformation lies not in its
presumed morphogenesis, but in the
underlying anatomy. An effective system
for describing this anatomy must be based
upon the morphology as it is observed. At
the same time, it must be capable of
accounting for all congenital cardiac
conditions, even those that, as yet, might
not have been encountered. To be useful
clinically, the system must be not only
broad and accurate, but also clear and
consistent. The terminology used,
therefore, should be unambiguous. It
should be as simple as possible. The
sequential segmental approach provides
such a system1, particularly when the
emphasis is placed on its surgical
applications2. The basis of the system is, in
the rst instance, to analyse individually
the architectural make-up of the atrial
chambers, the ventricular mass, and the
arterial segment3. Emphasis is thus given to
the nature of the junctional arrangements
(Figure 6.1). Still further attention is
devoted to the interrelationships of the
cardiac structures within each of the
individual segments. This provides the
icu
lar
Arterial
trunks
ria
rte
loa
icu
iov
Atr
The rst step in analysing any malformed

heart is to determine the arrangement of
the chambers within the atrial mass. When
distinction is based on the anatomy of the
appendages, which are the most constant
components of the atriums, and specically
on the extent of the pectinate muscles
relative to the atrial vestibules5, atrial
chambers can be of only morphologically
right or morphologically left type. The
morphologically right appendage is broad
and triangular (Figure 6.2), whereas the
morphologically left appendage is ngerlike, and has a much narrower neck
(Figure 6.3). In most instances, it is
possible to identify the appendages simply
on the basis of their shape (Figure 6.4).
Only in circumstances of uncertainty will it
prove necessary to inspect the extent of the
pectinate muscles (Figures 6.5, 6.6). This
feature, of course, is readily visible to the
surgeon once the atrial chambers have been
opened.
When judged on the extent of the
pectinate muscles, there are only four
topological ways in which the appendages
can be arranged within the atrial mass
(Figure 6.7). Almost always, the atrium
ntr
tr
en
ATRIAL ARRANGEMENT
possessing the appendage in which the

pectinate muscles extend to the crux is
right-sided, while the one with a smooth
inferoposterior vestibule is left-sided. This
usual arrangement is often called situs
solitus. Rarely, the appendages can be
disposed in mirror-image fashion, so-called
situs inversus. More common than the
mirror-imaged topological arrangement,
but still relatively rare, is the situation in
which the appendages of both chambers in
the atrial mass have the same morphology.
This can occur in two forms, with either
morphologically right (Figure 6.8) or
morphologically left (Figure 6.9)
appendages on both sides. These bilaterally
symmetrical topological patterns, or
isomeric arrangements, have traditionally
been named according to the arrangement
of the abdominal organs, particularly the
spleen. This is because they usually
exist with a jumbled up abdominal
arrangement, an arrangement also termed
visceral heterotaxy6. It is far more
convenient, as well as more accurate, to
designate them in terms of their own
intrinsic morphology8, particularly as this
can be determined readily by the surgeon
in the operating room. Isomerism of the
right appendages is usually, but not always,
found with absence of the spleen. It is most
often associated with right bronchial
isomerism. Isomerism of the left
appendages is typically found, but again
not always, with multiple spleens. The
Ve
Atriums
basic framework within which all other

associated malformations can be
catalogued4.
129
Fig. 6.1 The cartoon shows the three segments of the heart. These
Ventricles
are the atriums, the ventricular mass, and the arterial trunks. The
segments are joined together at the atrioventricular and
130
Triangular appendage
Sup.
Right
Left
Inf.
Fig. 6.2 The computed tomogram shows the typical triangular
shape of the morphologically right atrial appendage.
Hooked and tubular appendage
Sup.
Post.
Ant.
Fig. 6.3 As shown in this computed tomogram, the

Inf.
association with left bronchial isomerism is

more constant.
The anticipated topological
arrangement of the atrial appendages can
be predicted preoperatively with a high
morphologically left atrial appendage has a characteristic narrow

and hooked shape (compare with Figure 6.2).
degree of accuracy by studying the

relationships of the abdominal great vessels
as determined with cross-sectional
ultrasonography10. When identied,
knowledge of isomerism of the atrial
appendages is of value in two additional

ways. Firstly, it alerts the surgeon to
unusual dispositions of the sinus node11. In
right isomerism, the sinus node, being a
morphologically right atrial structure, is
131
Morphologically left appendage
Left
Sup.
Inf.
Right
Fig. 6.4 This operative view, taken through a median sternotomy,

shows the differences between the broad triangular
morphologically right atrial appendage, and the narrow nger-like
morphologically left atrial appendage.
Morphologically right appendage
Septum spurium
Sup.
Ant.
Post.
Inf.
Extensive pectinate muscles
Fig. 6.5 The computed tomogram shows the extensive pectinate

muscles found in the roof of the morphologically right atrium.
When assessed relative to the vestibule of the atrioventricular
junction, they extend to the crux of the heart.
duplicated. A node is found laterally in

each of the terminal grooves12. In left
isomerism, there are no terminal grooves
(Figure 6.9). In this situation, the sinus
node is a poorly formed structure, without
a constant site. Usually the node is found in

the anterior interatrial groove, close to the
atrioventricular junction12.
The second advantage of recognising
isomeric appendages is that the
arrangements are known to be harbingers

of complex intracardiac lesions. Hearts
with isomerism of either type tend to have
bilateral superior caval veins, an effectively
common atrial chamber, albeit with two
132
Pulmonary veins
Sup.
Post.
Ant.
Fig. 6.6 As shown in this computed tomogram, the pectinate

Pectinate muscles
Inf.
Mirror-imaged
Usual
Isomeric right
Isomeric left
isomeric appendages, and common

atrioventricular valves. Right isomerism is
always associated with a totally anomalous
pulmonary venous connection, even if the
pulmonary veins are joined to one or other
atrium. It is also seen most frequently with
pulmonary stenosis or atresia, and in
association with a univentricular
atrioventricular connection, typically a
double-inlet ventricle through a common
valve. Left isomerism, in the majority of
cases, is associated with interruption of the
muscles of the morphologically left appendage are conned within

the tubular component, which has a very narrow junction with the
body of the atrium (black double-headed arrow).
Fig. 6.7 The cartoon shows the four possible arrangements of the
atrial appendages, which cannot always be distinguished on the
basis of their shape. The best means of distinguishing between
them is to establish the extent of the pectinate muscles. These
muscles extend all the way to the crux in the morphologically right
atrial appendage, but are conned around the mouth of the
appendage in the morphologically left atrial appendage, leaving a
smooth posterior vestibule. Using this criterion, all congenitally
malformed hearts have appendages tting within one of the four
groups shown in the cartoon.
inferior caval vein, with continuation of the

venous drainage from the abdomen
through the azygos system of veins.
THE ATRIOVENTRICULAR
JUNCTIONS
Having established the arrangement of the
atrial appendages, the next step in
sequential analysis is to determine the
morphology of the atrioventricular
junctions. For this, the surgeon needs to

know how the atrial chambers are, or are
not, connected to the chambers present
within the ventricular mass. Most often,
there are two such chambers, which can be
of only right or left morphology. The
morphological distinction is based on the
nature of the apical trabeculations. In the
morphologically right ventricle, these
trabeculations are coarse, in contrast to the
ne criss-crossing trabeculations that
characterise the morphologically left
133
Left-sided morphologically right appendage
Fig. 6.8 This surgical view through a median sternotomy
Left
Inf.
Sup.
Right
shows a left-sided atrial appendage of morphologically right

pattern. The right-sided appendage is also of right morphology,
so the patient has isomerism of the right atrial appendages. Note
the crest of the appendage in relation to the left superior caval vein
and the terminal groove. When examined internally, pectinate
muscles encircled both atrioventricular junctions.
Left
Sup.
Inf.
Right
Fig. 6.9 This surgical view through a median sternotomy shows a
Right-sided morphologically left appendage
ventricle (Figure 6.10). It is the

interrelationships between the two
ventricles that permit the description of
ventricular topology. When analysed
according to the way that the palmar
surfaces of the hands can be placed on the
right-sided atrial appendage of morphologically left pattern. The

left-sided appendage is also of left morphology, so the patient has
isomerism of the left atrial appendages. Note the absence of any
terminal groove. Internal inspection conrmed the presence of
smooth bilateral posterior vestibules.
septal surface of the morphologically right

ventricle such that the thumb is within the
inlet component, and the ngers in the
ventricular outlet, the patterns reect
either right-hand or left-hand topology
(Figure 6.11). Having assessed ventricular
morphology, it is possible to determine the

way in which the atrial chambers are
connected, or not connected, to the
ventricular mass. In most instances, there
are two atrioventricular junctions, although
one of the junctions can be absent. Also
134
Left
Post.
Coarse right
ventricular
trabeculations
Ant.
Right
Fig. 6.10 The apical part of the normal ventricular mass has been
amputated, and is viewed from above. It shows the marked
difference between the ne apical trabeculations of the
morphologically left ventricle when compared to the coarse right
ventricular apical trabeculations.
Fine left ventricular trabeculations
Fig. 6.11 The cartoon shows how the
Right-hand topology
Left-hand topology
patterns of ventricular topology can be

described, guratively speaking, in terms of
the way that the palmar surface of the hands
can be placed on the septal surface of the
morphologically right ventricle. The ngers
point up the outlet, and the thumb lies in the
inlet, giving right-hand and left-hand patterns.
In the arrangements shown, the
atrioventricular connections are concordant,
but the ventriculoarterial connections can also
be discordant, with the aorta arising from the
morphologically right ventricle (see
Figure 6.29).
important is the morphology of the valves

that guard the atrioventricular junctions,
because the paired junctions can be
guarded by a common atrioventricular
valve. This shows that junctional and valvar
morphology are separate and independent
features.
There are ve distinct and discrete ways
in which the atrial chambers may be
connected to the ventricular mass, the nal
one having two subtypes, along with an
intriguing further variation. Most often,

the atrial chambers are connected to their
morphologically appropriate ventricles.
This pattern is called concordant
atrioventricular connections. When each
atrium is connected in this way to its own
ventricle, there is rarely any difculty in
distinguishing the morphology of the
ventricles, even when the ventricles
themselves are unusually related one to the
other. In the second pattern, which
represents discordant connections, each

atrium is connected with a morphologically
inappropriate ventricle.
Concordant and discordant
connections can exist with either the usual
or mirror-imaged arrangement of the
atrial appendages (Figure 6.12), but not
with isomeric appendages. When the
appendages are isomeric, and each atrium
is connected to its own ventricle, then of
necessity one junction will be
Usual arrangement
Mirror-imaged pattern
Concordant
Concordant
135
Fig. 6.12 The cartoon shows how the atrial chambers can be
Discordant
Discordant
Right isomerism
Left isomerism
Right-hand topology
Right-hand topology
connected to the ventricles in concordant or discordant fashion,

with each pattern existing in usual and mirror-imaged variants.
Fig. 6.13 The cartoon demonstrates the mixed and biventricular
Left-hand topology
Left-hand topology
concordantly connected, but the other

junction will be discordantly connected
(Figure 6.13). This will occur irrespective
of the topological pattern of the
atrioventricular connections found when there are isomeric

atrial appendages, and each atrium is connected to its own
ventricle. In each pattern, half of the heart is concordantly
connected, and the other half is discordant. It is essential in these
settings, therefore, to describe both the type of isomerism, and the
specic ventricular topology.
ventricular mass (see later). The

arrangement produces a third discrete
pattern, namely biventricular and mixed
atrioventricular connections.
In the three connections described

thus far, each atrium is connected to its
own ventricle. This means that the
atrioventricular connections themselves
136
are biventricular. The essential feature in

the remaining two types of
atrioventricular connection is that the
atrial chambers, with one exception,
connect to only one ventricle. In one of
these patterns, both atrial chambers
connect to the same ventricle. This is a
double-inlet atrioventricular connection
(Figure 6.14). In the other variant, one of
the atrial chambers is connected to a
ventricle, but the other atrium has no
connection with the ventricular mass.
This latter arrangement can be divided
into two subtypes, depending on whether
absence of the connection is right-sided
(Figure 6.15) or left-sided (Figure 6.16).
An intriguing variation is seen when one
of the atrioventricular connections is

absent, be it right-sided or left-sided,
namely when the atrioventricular valve
guarding the solitary connection straddles
the septum, being attached in both
ventricles. The end result is a uniatrial,
but biventricular, atrioventricular
connection (Figure 6.17).
There has been much controversy
concerning the description of the hearts
in which the atrial chambers connect to
only one ventricle. It became conventional
to describe them in terms of single
ventricle, common ventricle, or
univentricular hearts. It is exceedingly rare,
however, to nd patients with solitary
ventricles. Almost always, in patients
described as having univentricular hearts,

the ventricular mass contains more than
one chamber. By focusing on the fact that
the atrioventricular connection is, in
reality, joined to only one ventricle, we are
able to achieve a satisfactory solution for
this dilemma. Thus, the hearts can logically
and accurately be described in terms of
being functionally univentricular. It
follows that, in some patients with
biventricular atrioventricular connections,
imbalance between the ventricles can again
produce a functionally univentricular
arrangement13. In those with
univentricular atrioventricular
connections, however, one of the ventricles
must be incomplete, while the other
Left atrium
Right atrium
Dominant left ventricle
Sup.
Right
Left
Fig. 6.14 When both atriums are connected to only one ventricle,
Inf.
the atrioventricular connection is univentricular. In this heart,

showing a four-chamber section in anatomical orientation, there is
a double inlet to a dominant left ventricle. The black braces show
the segments of atrial vestibular myocardium, connected to the
dominant left ventricle through separate atrioventricular valves.
137
Sup.
Right
Left atrium
Left
Inf.
Fig. 6.15 This anatomical specimen, seen in

Right atrium
Right AV groove
four-chamber orientation, shows the absence

of the right atrioventricular connection, with
the broadipose tissue of the right
atrioventricular (AV) groove interposing
between the right atrial oor and the base of
the ventricular mass. This arrangement
produces another form of univentricular
atrioventricular connection. In this case, the
morphology is that of classical tricuspid atresia,
with the left atrium connected to a dominant
left ventricle. Note the presence of the base of
the incomplete right ventricle (arrow), which
has no connection with the atrial chambers.
Left atrium
Right atrium
Sup.
Fig. 6.16 This anatomical specimen, again seen in four-chamber

Right
Left
orientation (compare with Figures 6.14 and 6.15), shows the

absence of the left atrioventricular connection (red dotted line),
giving the third variant of univentricular atrioventricular
connection. In this example, the right atrium is connected to a
dominant left ventricle through a right-sided atrioventricular valve.
Inf.
ventricle is dominant. The dominant

ventricle, which supports the
atrioventricular junction or junctions, can
take one of three morphologies: right, left,
or indeterminate (Figure 6.18). Most
frequently, as judged from the pattern of its
apical trabecular component, the dominant

ventricle will be morphologically left.
There will be a complementary right
ventricle, perforce incomplete because it
will lack its atrioventricular connection,
and hence its inlet component. Such
incomplete right ventricles are always

found anterosuperiorly relative to the
dominant left ventricle, irrespective of
whether there is a double inlet, or an absent
right or absent left atrioventricular
connection. They can, however, be
138
Straddling & overriding AV valve
LA
RA
RV
Fig. 6.17 This cartoon illustrates the arrangement when there is an

absence of the right atrioventricular (AV) connection, but with the
solitary atrioventricular valve straddling the ventricular septum. This
produces a uniatrial but biventricular atrioventricular connection,
shown here in the setting of a usual atrial arrangement with righthand ventricular topology. RA, LA, right and left atriums; RV, LV,
right and left ventricles.
LV
Absent AV connection
Usual
Mirror-imaged
Right isomerism
Left isomerism
Fig. 6.18 This cartoon shows the multiple possibilities for
Dominant left with

incomplete RV
Solitary and
indeterminate
ventricle
positioned either to the right (Figure 6.19),

or to the left (Figure 6.20) relative to the
dominant ventricle.
More rarely, the atrial chambers can be
connected to a dominant right ventricle.
This happens most frequently in the
absence of the left atrioventricular
Dominant right
with incomplete LV
univentricular atrioventricular connection that can be produced

by combining the variations in atrial morphology, atrioventricular
connection, and ventricular morphology. It takes no account of
the further variations possible according to ventricular
relationships, ventriculoarterial connections, etc. The arrows in
the middle row emphasise the absence of one of the
atrioventricular connections. LV, RV, left and right ventricles.
connection (Figure 6.21), but can be found

with a double inlet or, rarely, with an
absence of the right atrioventricular
connection. When only the right ventricle
is connected to the atrial chambers, and
hence dominant, it is the left ventricle that
is incomplete, lacking its atrioventricular
connection and its inlet portion. The

incomplete left ventricle will always be
found in a posteroinferior position. Usually
it is left-sided, although rarely it can be
right-sided.
The third morphological
conguration found with a double inlet
139
Sup.
Aorta
Left
Right
Inf.
VSD
Fig. 6.19 In this anatomical specimen with a double-inlet left

ventricle, the incomplete right ventricle is positioned
anterosuperiorly and to the right of the dominant left ventricle. VSD,
ventricular septal defect.
Incomplete right ventricle
Sup.
Right
Left
Aorta
Inf.
VSD
Fig. 6.20 This anatomical specimen, again with a double-inlet left

ventricle, has the incomplete right ventricle in an anterosuperior
and leftward position relative to the dominant left ventricle. VSD,
or, exceedingly rarely, with an absence of

either atrioventricular connection, is
when the atrial chambers connect to a
solitary ventricle. Such solitary
ventricles have indeterminate apical

trabecular morphology (Figure 6.22).
Incomplete second ventricles are
never found in this variant of
univentricular atrioventricular
connection, in which the only septal
structure in the ventricle is that
separating the outow tracts.
140
Incomplete left ventricle
Left
atrium
Right
atrium
Dominant right ventricle
Fig. 6.21 This section through a specimen,
Sup.
shown in four-chamber orientation, illustrates

the absence of the left atrioventricular
connection (red dotted line) with the right
atrium connected to a dominant right
ventricle. This produces one of the variants of
the hypoplastic left heart syndrome.
Left
Right
Inf.
To pulmonary trunk
Coarse apical
trabeculations
Left AV
valve
To aorta
Sup.
Right AV
valve
Fig. 6.22 This heart, opened in

Left
Right
Inf.
VALVAR MORPHOLOGY
The atrioventricular valvar morphology is
independent of the way in which the atrial
chambers connect with the ventricles. Valvar
morphology, therefore, constitutes a separate
feature of the atrioventricular junctions.

When there are concordant, discordant,
mixed, or double-inlet connections, then
both atrial chambers are connected, actually
or potentially, to the ventricular mass. The
two atrioventricular junctions can be
clam-shell-like fashion, has a double inlet to,

and double outlet from, a solitary and
indeterminate ventricle, the ventricle itself
having very coarse apical trabeculations. AV,
atrioventricular.
guarded by two separate atrioventricular

valves (Figure 6.23), or by a common valve
(Figure 6.24). When there are two valves,
either of them can be imperforate, blocking a
potential atrioventricular connection. An
imperforate valve, therefore, needs to be
141
Base
Left
Right
Apex
Fig. 6.23 This normal heart, sectioned in

four-chamber orientation, shows the right and
left atrioventricular junctions (black dotted
lines with double-headed arrows) guarded by
separate atrioventricular valves.
Left
atrium
Right
atrium
Left
ventricle
Right
ventricle
Sup.
Left
Right
Fig. 6.24 This section, again in four-chamber orientation

(compare with Figure 6.23), shows that the right and left
atrioventricular junctions (black dotted line with double-headed
arrow) are guarded by a common atrioventricular valve in the
setting of an atrioventricular septal defect.
Inf.
distinguished from absence of an

atrioventricular connection, as either can
produce atrioventricular valvar atresia.
The essence of the imperforate valve is that
the atrioventricular connection has formed,

but is blocked by the conjoined valvar
leaets (Figure 6.25). In the setting of
absence of the connection, the oor of the
atrium involved is completely separated

from the ventricular mass by the
broadipose tissue of the atrioventricular
groove (see Figures 6.15, 6.16).
142
Sup.
Left
Right
Inf.
Left
atrium

Right
atrium
Fig. 6.25 This section of a heart, cut in four-chamber orientation,

shows an imperforate right atrioventricular valve connecting to
a hypoplastic right ventricle. The atrioventricular connections
remain concordant, even though the right valve (arrow) is
imperforate.
Hypoplastic right ventricle
Left atrium
Right atrium
Base
Fig. 6.26 This section of a heart, seen in four-chamber

orientation, shows how the tension apparatus of the right
atrioventricular valve (arrows) is attached to both sides of the
ventricular septum (star), while the orice of the right
atrioventricular junction (white brace) is overriding the septal
crest (star).
Left
Right
Apex
Either of two valves, or a common

valve, can also straddle a septum within the
ventricular mass. Straddling of the tension
apparatus of a valve should be distinguished
from overriding of its supporting
atrioventricular junction. Straddling exists
when the valvar tension apparatus is
attached to both sides of the ventricular

septum (Figure 6.26). Overriding is present
when the junction is connected to both
ventricles. The degree of override, which
usually coexists with straddling, determines
the precise atrioventricular connection
present. So as to adjudicate the connection
in the presence of overriding, the overriding

valve is assigned to the ventricle
connected to its greater part (Figure 6.27).
The possible arrangements are much
more limited when one atrioventricular
connection is absent. In this situation, the
solitary valve can either be committed in its
143
Fig. 6.27 In the presence of an

RA
LA
RA
LA
RV
RV
LV
LV
Straddling tricuspid
valve with doubleinlet left ventricle
Straddling
tricuspid valve
concordant AV
connections
entirety to one ventricle, or it can straddle

and override. When the valve straddles and
overrides in this setting, then the
atrioventricular connection itself is
uniatrial but biventricular (Figure 6.17).
VENTRICULAR MORPHOLOGY
AND TOPOLOGY
The nature of the atrioventricular
connections is inextricably linked with the
architectural arrangement of the
ventricular mass. Biventricular
atrioventricular connections, for example,
cannot be diagnosed without knowledge of
ventricular morphology. Double-inlet and
absent connections can all be identied
without mention of ventricular
morphology, although in this setting it is
always necessary to give more information
concerning the arrangement of the
ventricular mass. In the case of mixed and
biventricular atrioventricular connections,
it is important to describe the pattern in
which the morphologically right ventricle
is structured relative to the
morphologically left ventricle. This feature
can take only one of two topological
arrangements. This is because, when the
connections are mixed, the right-sided
atrium, with either a morphologically right
or left appendage, can be connected to

either a morphologically right or a
morphologically left ventricle (see
Figure 6.13). When there is right
isomerism, and the right-sided atrium is
connected to a morphologically right
ventricle, the ventricular mass typically is
seen as in hearts with concordant
atrioventricular connections and the usual
atrial arrangement. In contrast, when there
is right isomerism, and the right-sided
atrium is connected to a morphologically
left ventricle, the ventricular mass is
usually found in the presence of discordant
atrioventricular connections and the usual
atrial arrangement.
As we have discussed already, these two
basic patterns of ventricular topology can
conveniently be described according to the
way in which the hands, guratively
speaking, can be placed palm downwards
upon the septal surface of the
morphologically right ventricle. The other
hand will then t in the morphologically
left ventricle in similar fashion, but it is the
arrangement of the morphologically right
ventricle that is chosen for the purposes of
description (Figure 6.11). In the hearts
with biventricular and mixed
atrioventricular connections, it is this
ventricular topology that determines the
disposition of the atrioventricular
overriding junction, whether it is a straddling

tricuspid valve as shown in Figure 6.26 or
ventriculoarterial, the overriding junction
(black braces) is assigned to the ventricle
supporting its greater part. In the situation
illustrated with the straddling tricuspid
valve, the atrioventricular connections are
dened accordingly. There is a spectrum
between the illustrated extremes (doubleheaded arrow). The left-hand panel shows the
situation with the junction connected primarily
to the right ventricle (large arrow), the lesser
part joining the left ventricle (small arrow).
Hence, the atrioventricular connections are
deemed to be concordant. In the right-hand
panel, the larger part of the overriding
junction is committed to the left ventricle, so
that the connection is deemed to be a double
inlet. This is the essence of the 50% rule. RA,
LA, right and left atriums; RV, LV, right and left
ventricles.
conduction tissues11. When the atrial

chambers connect to only one ventricle, the
morphology of that ventricle must always
be described. This is because the dominant
ventricle can be of left ventricular, right
ventricular, or solitary and indeterminate
pattern. It is also necessary to describe the
relationships of the dominant and
incomplete ventricles.
VENTRICULAR RELATIONSHIPS
Ventricular relationships, as opposed to
ventricular topology, generally should be
described as a separate feature of the heart.
Where each atrium is connected to its own
ventricle, the relationships are almost
always in harmony with both the
connection and topology present. When
the atrial chambers are in their usual
position with concordant atrioventricular
connections, the relationships described in
the setting of the heart within the chest are
almost always for the morphologically right
ventricle to be right-sided, anterior, and
inferior to the morphologically left
ventricle. In mirror-imaged atrial
arrangement, with concordant
atrioventricular connections, the
morphologically right ventricle is almost
invariably left-sided and relatively anterior,
144
Sup.
Aorta
Post.
Ant.
Inf.
Outlet
Fig. 6.28 The left side of the heart is shown from a patient with
Inlet
discordant atrioventricular and ventriculoarterial connections; in

other words, congenitally corrected transposition. It is the palmar
surface of the left hand only that can be placed on the septal
surface of the morphologically right ventricle so that the ngers
occupy the ventricular outlet, and the thumb goes in the tricuspid
valve guarding the inlet component.
Morph. right apical trabeculations
Morph. left atrium

Aorta
Outlet
Inlet
Fig. 6.29 The computed tomogram shows the right side of the
Sup.
Ant.
Post.
Inf.
but frequently more side-by-side relative to

its neighbour.
With the atrial chambers in their usual
arrangement, and discordant atrioventricular
connections, there is almost always the lefthand pattern of ventricular topology. The
usual relationship is for the morphologically
heart from a patient having congenitally corrected transposition in

the setting of a mirror-imaged atrial arrangement. The
morphologically left atrium is right-sided, but connects to a
morphologically right ventricle with right-hand topology, the
palmar surface of the right hand tting on the septal surface so
that the ngers are in the subaortic outlet and the thumb in the
inlet component.
right ventricle to be left-sided (Figure 6.28).

When discordant atrioventricular
connections accompany mirror-imaged atrial
arrangement, there is usually right-hand
topology (Figure 6.29). The ventricular
relationships are thus similar to those seen in
the normal heart, although the two chambers
tend to be more side-by-side. When the

relationships are as anticipated, it is
unnecessary to describe them. Very
occasionally, the relationships of the
ventricles are not as anticipated for the
connections present. This disharmony
between connections and relationships
145
Fig. 6.30 This cartoon shows the patterns
Aorta
Pulmonary
trunk
Common
arterial trunk
underscores the anomaly known as the crisscross heart14. With these hearts, and also
those with superoinferior ventricles,
connections and relationships must be
described separately, using as much detail as
is necessary to achieve unambiguous
categorisation. The essence of the criss-cross
heart, and those with superoinferior
ventricles, is that the ventricular
relationships are not as expected for the
atrioventricular connection present. Even
more rarely, the ventricular topology may be
disharmonious with the atrioventricular
connection15. All features must then be
described.
In hearts with a univentricular
atrioventricular connection, it is the
relationship of the incomplete ventricle to
the dominant ventricle that must be
described. When the left ventricle is
dominant, the incomplete right ventricle is
always anterosuperior, but can be right- or
left-sided. The sidedness of the ventricle
does not affect the basic disposition of the
atrioventricular conduction tissues in these
hearts. With a dominant right ventricle, the
incomplete and rudimentary left ventricle,
if present, is always posteroinferior, but
again can be right- or left-sided. In this
case, the sidedness of the incomplete
ventricle will affect the disposition of the
atrioventricular conduction tissue.
When considering the atrioventricular
junctions, therefore, there are four
different features to take into account.
These are, rstly, the way the atrial
myocardium is connected to the ventricular
mass; secondly, the morphology of the
atrioventricular valves guarding the
junctions; thirdly, the ventricular
morphology and topology; and nally, the
Solitary
arterial trunk
ventricular relationships. All are of

importance to the surgeon because they
inuence the disposition of the
atrioventricular conduction axis.
VENTRICULOARTERIAL
JUNCTIONS
Analysis of the ventriculoarterial
junctions proceeds as described for the
atrioventricular junctions, with the
morphology of the connections, the
valvar morphology, and the relationships
of the arterial trunks being different
facets requiring separate description in
mutually exclusive terms. It is also
necessary to take account of infundibular
morphology.
Ventriculoarterial connections
There are four discrete ways in which the
arterial trunks can take their origin from
the ventricular mass; namely, in
concordant, discordant, double-outlet, and
single-outlet fashion. Concordant
ventriculoarterial connections exist when
the arterial trunks arise from
Discordant connections account for the
trunks being connected with
morphologically inappropriate ventricles.
Double-outlet connections exist when both
great arteries take origin from the same
ventricle, which may be of right, left, or
indeterminate morphology. A single-outlet
arrangement is seen when only one arterial
trunk is connected to the heart. This may
be a common trunk, directly supplying the
systemic, pulmonary, and coronary
that identify the morphology of the arterial

trunks. When there is absence of the
intrapericardial pulmonary arteries, there is no
way of knowing if, had there been an atretic
pulmonary trunk, it would have originated
from the base of the heart or from the aorta.
This pattern, therefore, is best described as a
solitary arterial trunk.
arteries, or it may be an aortic or pulmonary

trunk when the complementary arterial
trunk is atretic, and its connection to a
known ventricle cannot be established
(Figure 6.30). Rarely, in the absence of
intrapericardial pulmonary arteries, it may
be more accurate to describe an arterial
trunk as solitary rather than common
(Figure 6.30).
Arterial valvar morphology

The morphological arrangement of the
arterial valves is limited because they have
no tension apparatus. Furthermore, a
common valve can exist only with a
common trunk. The different patterns,
therefore, involve one or two arterial
valves. Usually both valves are perforate,
but either or both may override the
ventricular septum. When a valvar orice is
overriding, the valve is assigned to the
ventricle supporting its greater part, thus
avoiding the need for intermediate
categories. The other pattern of valvar
morphology is when one of the arterial
valves is imperforate. As with the
atrioventricular junctions, an imperforate
arterial valve must be distinguished from
absence of one ventriculoarterial
connection, as both can produce arterial
valvar atresia.
Infundibular morphology
Describing the morphology at the
ventriculoarterial junctions also involves
the arrangement of the musculature within
the ventricular outow tracts. This is
infundibular morphology. Although the
outlet regions are integral parts of the
146
Pulmonary
valve
Outlet
septum
Aortic
valve
SMT
Sup.
Left
Right
Inf.
Interventricular communication
Outlet septum
Fig. 6.31 This view of a heart with a double-outlet right ventricle,

seen in anatomical orientation, and with a non-committed
interventricular communication, shows the muscular components
of the ventricular outow tracts. The hinges of both arterial valves
are completely surrounded by outow musculature. SMT,
septomarginal trabeculation.
Pulmonary valve
Parietal
attachment
Septal
attachment
Sup.
Ant.
Post.
Inf.
ventricular mass, it is traditional to

consider them in concert with the great
arteries. The two outow tracts together
make a complete cone of musculature,
Fig. 6.32 The image shows the outow tracts in a heart with
tetralogy of Fallot, viewed from the apex of the right ventricle,
with the aortic valve (star) overriding the crest of the muscular
ventricular septum, which is reinforced by the septomarginal
trabeculation, or septal band (white Y). The septal and parietal
attachments of the muscular outlet septum are well seen.
which has parietal and septal components,

along with a component adjacent to the
atrioventricular junction (Figure 6.31).
The parietal components make up the
anterior free wall of the outow tracts. The

septal component is the outlet, or
infundibular, septum. This has a body,
with septal and parietal insertions, best
Outlet
septum
Aortic
valve
Sup.
Post.
Fig. 6.33 In this heart with tetralogy of Fallot, the

Ant.
Inf.
Aortic
valve
SMT
Outlet
septum
ventriculoinfundibular fold (red dotted line) interposes between

the leaets of the aortic and mitral valves in the roof of the
interventricular communication (star), producing a completely
muscular subaortic infundibulum. SMT, septomarginal
trabeculation.
Pulmonary
valve
Tricuspid
valve
Sup.
Post.
Ant.
Inf.
seen in the setting of tetralogy of Fallot

(Figure 6.32). The component adjacent to
the atrioventricular junction is the
ventriculoinfundibular fold. It separates
the leaets of arterial from atrioventricular
valves (Figure 6.33). It is the inner heart
curvature16. The fold may be attenuated to
Fig. 6.34 This heart has the ventriculoarterial connection of a

double-outlet right ventricle, but with brous continuity between
the leaets of the aortic and tricuspid valves (red double-headed
arrow).
give arterial-atrioventricular valvar brous

continuity (Figure 6.34).
The infundibular structures never
contain or overlie conduction tissue17.
They should be distinguished from
another structure, namely the
septomarginal trabeculation, or septal
band. This latter structure is part of the

ventricular septum, reinforcing its right
ventricular aspect. It has a body that
continues apically as the moderator band,
and two limbs (Figure 6.35). The
anterocephalad limb, in the normal
heart, extends to the pulmonary valve,
147
148
Pulmonary
valve
Tricuspid
valve
Sup.
Fig. 6.35 The septal surface of the normal right ventricle is

photographed to show the extent of the septomarginal
trabeculation, or septal band, as shown by the white Y. In the
normal heart, the supraventricular crest inserts between the limbs
of the trabeculation. The star shows the moderator band, one of
the series of septoparietal trabeculations that take origin from the
anterior surface of the marginal trabeculation.
Left
Right
Inf.
overlying the outlet septum. The

posterocaudal limb runs beneath the
interventricular membranous septum.
This posterocaudal limb usually overlies
the branching part of the atrioventricular
bundle, with the right bundle branch
passing down to the apex of the right
ventricle within the body of the
trabeculation.
Although each outow tract is
potentially a complete muscular structure,
in most hearts it is only the outow tract of
the right ventricle that is a complete
muscular cone. This is because, within the
left ventricle, part of the
ventriculoinfundibular fold is usually
attenuated to permit brous continuity
between the leaets of the arterial and
atrioventricular valves. In the normal
heart, therefore, there is a muscular
subpulmonary infundibulum in the right
ventricle, and brous valvar continuity in
the roof of the left ventricle. In
congenitally malformed hearts, three
other patterns may be found. These are,
rstly, a muscular subaortic infundibulum
with pulmonary-atrioventricular
continuity; secondly, a bilaterally
muscular infundibulum; and thirdly,
bilateral deciency of the infundibulums.
In the presence of a common arterial

trunk, there may be a complete muscular
subtruncal infundibulum, but more often
there is truncal-atrioventricular valvar
continuity.
Arterial valvar and truncal

relationships
The nal feature of consideration at the
ventriculoarterial junctions is the
relationship of the arterial valves and
arterial trunks. Valvar relationships are
independent of both ventriculoarterial
connections and infundibular
morphology. Of the many methods of
description, our preference is to describe
the aortic relative to the pulmonary valve
as viewed from below in rightleft,
anteroposterior and, when necessary,
superoinferior coordinates. This can be
done as precisely as required. In our
experience, eight coordinates combining
lateral and anterior to posterior positions
sufce (Figure 6.36). When describing the
relationship of the arterial trunks, it is
sufcient to account for trunks that spiral
around each other as they ascend, and to
distinguish them from trunks that ascend
in parallel fashion18.
POSITION OF THE HEART

The system discussed in this chapter
establishes the cardiac template.
Irrespective of the internal architecture, it
is well known that the heart itself can
occupy many and varied positions (see
Chapter 10), particularly when there
are complex intracardiac malformations.
To describe the position of the heart in
unambiguous fashion, account should
be taken separately of its site within
the thorax, and the orientation of its
apex. We describe the heart as being in
the left or right side of the chest, or in
the midline. Apical orientation is described
as being to the left, to the middle, or to
the right.
CATALOGUE OF
MALFORMATIONS
Having described the template of the heart,
and its position, nally it is necessary to
catalogue all intracardiac malformations. In
most cases, it is these lesions which will
require surgical attention. Any lesion,
nonetheless, cannot be presumed to be the
only lesion present until the rest of the
149
anterior
right
anterior
left
anterior
left
side-by-side
right
side-by-side
Pulmonary trunk
right
posterior
normal
left
posterior
Fig. 6.36 The cartoon shows the combination of anterior to

posterior, and right to left coordinates that are used to describe the
interrelationships of the aortic and pulmonary valves at their
origins from the ventricular mass.
posterior
heart has been established as normal. It is

these associated lesions that will be
emphasised in subsequent chapters, taking
particular note, as before, of the features of
surgical signicance.
6.
References cited
1. Shinebourne EA, Macartney FJ, Anderson
RH. Sequential chamber localization:
the logical approach to diagnosis in
congenital heart disease. Br Heart J 1976; 38:
327340.
2. Anderson RH, Wilcox BR. Understanding
cardiac anatomy: the prerequisite for optimal
cardiac surgery. Ann Thorac Surg 1995; 59:
13661375.
3. Van Praagh R. The segmental approach to
diagnosis in congenital heart disease. In:
Bergsma D (ed). Birth defects original article
series, Vol. VIII, No. 5. The Fourth
Conference on the Clinical Delineation of Birth
Defects. Part XV The Cardiovascular System.
The National Foundation March of Dimes.
Baltimore, MD: Williams and Wilkins, 1972;
pp 423.
4. Anderson RH, Ho SY. Continuing Medical
Education. Sequential segmental analysis
description and categorization for the
millennium. Cardiol Young 1997; 7:
98116.
5. Uemura H, Ho SY, Devine WA, Kilpatrick
LL, Anderson RH. Atrial appendages and
7.
8.
9.
10.
venoatrial connections in hearts with

patients with visceral heterotaxy. Ann
Thorac Surg 1995; 60: 561569.
Van Mierop LHS, Gessner IH, Schiebler
GL. Asplenia and polysplenia syndromes.
In: Bergsma D (ed). Birth Defects: Original
Article Series, Vol VIII, No 5. The Fourth
Conference on the Clinical Delineation of
Birth Defects. Part XV The Cardiovascular
System. The National Foundation March of
Dimes. Baltimore, MD: Williams and
Wilkins, 1972: 3644.
Ivemark BI. Implications of agenesis of
the spleen on the pathogenesis of
conotruncus anomalies in childhood. An
analysis of the heart; malformations in
the splenic agenesis syndrome, with 14
new cases. Acta Paediatr Suppl 1955; 44:
7110.
Macartney FJ, Zuberbuhler JR, Anderson
RH. Morphological considerations
pertaining to recognition of atrial
isomerism. Consequences for sequential
chamber localisation. Br Heart J 1980; 44:
657667.
Sharma S, Devine W, Anderson RH,
Zuberbuhler JR. The determination of
atrial arrangement by examination of
appendage morphology in 1842 heart
specimens. Br Heart J 1988; 60: 227231.
Huhta JC, Smallhorn JF, Macartney FJ.
Two dimensional echocardiographic
diagnosis of situs. Br Heart J 1982; 48:
97108.
11. Smith A, Ho SY, Anderson RH, et al. The

diverse cardiac morphology seen in hearts
with isomerism of the atrial appendages
with reference to the disposition of the
specialized conduction system. Cardiol
Young 2006; 16: 437454.
12. Ho SY, Seo J-W, Brown NA, et al.
Morphology of the sinus node in human
and mouse hearts with isomerism of the
atrial appendages. Br Heart J 1995; 74:
437442.
13. Jacobs ML, Anderson RH. Nomenclature
of the functionally univentricular heart.
Cardiol Young 2006; 16(Suppl 1): 38.
14. Anderson RH. Criss-cross hearts revisited.
Pediatr Cardiol 1982; 3: 305313.
15. Anderson RH, Smith A, Wilkinson JL.
Disharmony between atrioventricular
connections and segmental combinations
unusual variants of criss-cross hearts.
J Am Coll Cardiol 1987; 10: 12741277.
16. Anderson RH, Becker AE, Van Mierop
LHS. What should we call the crista? Br
Heart J 1977; 39: 856859.
17. Hosseinpour A-R, Jones TJ, Barron DJ,
Brawn WJ, Anderson RH. An appreciation
of the structural variability in the
components of the ventricular outlets in
congenitally malformed hearts. Eur J
Cardiothorac Surg 2007; 31: 888893.
18. Cavalle-Garrido T, Bernasconi A, Perrin
D, Anderson RH. Hearts with concordant
ventriculoarterial connections but parallel
arterial trunks. Heart 2007; 93: 100106.
Lesions with
normal segmental
connections
Normal segmental connections
SEPTAL DEFECTS
Understanding the anatomy of septal
defects is greatly facilitated if the heart
is thought of as having three distinct
septal structures: the atrial septum,
the atrioventricular septum, and the
ventricular septum (Figure 7.1). The
normal atrial septum is relatively small. It is
made up, for the most part, by the oor
of the oval fossa. When viewed from the
right atrial aspect, the fossa has a oor,
surrounded by rims. The oor is derived
from the primary atrial septum, or septum
primum. Although often considered to
represent a secondary septum, or septum
secundum, the larger parts of the rims,
specically the superior, anterosuperior,
and posterior components, are formed by
infoldings of the adjacent right and left
atrial walls. Inferoanteriorly, in contrast,
the rim of the fossa is a true muscular
septum (Figure 7.2). This part of the rim is
contiguous with the atrioventricular
septum, which is the superior component
of the brous membranous septum. In the
normal heart, this brous septum is also
contiguous with the atrial wall of the
triangle of Koch (Figure 7.3). In the past,

we considered this component of the atrial
wall, which overlaps the upper part of the
ventricular musculature between the
attachments of the leaets of the tricuspid
and mitral valves, as the muscular
atrioventricular septum. As we discussed in
Chapter 2, we now know that it is better
viewed as a sandwich1. This is because,
throughout the oor of the triangle of
Koch, the broadipose tissue of the inferior
atrioventricular groove separates the layers
of atrial and ventricular myocardium
(Figure 7.4). From the stance of
understanding septal defects, nonetheless,
it is helpful to consider the entire area
comprising the brous septum and the
muscular sandwich as an atrioventricular
separating structure, as it is absent in the
hearts we describe as having
atrioventricular septal defects.
The ventricular septum is usually seen by
the surgeon only from its right ventricular
aspect. For this and other reasons we will
discuss, holes between the ventricles are best
considered in terms of their right ventricular
landmarks. Taken overall, the ventricular
septum is made up of a small brous
151
element, specically the interventricular

part of the membranous septum, and a much
larger muscular part. The muscular part,
which is signicantly curved, is more
complex geometrically than the other septal
structures, which lie almost completely in
the coronal plane. At rst sight, it seems
possible to divide the muscular septum into
inlet, apical trabecular, and outlet
components, each of these parts seemingly
corresponding with the components of the
right ventricle, and abutting centrally on the
membranous septum (Figure 7.5). Closer
inspection shows that such analysis is
simplistic. By virtue of the deeply wedged
location of the subaortic outow tract, much
of the septum delimited on the right
ventricular aspect by the septal leaet of
the tricuspid valve separates the inlet of the
right ventricle from the outlet of the left
(Figure 7.6). The muscular wall forming the
back of the subpulmonary infundibulum is,
at rst sight, an outlet septum. Only a
small part of this wall, however, interposes
between the cavities of the right and left
ventricles. This is because most of the
subpulmonary infundibulum is a freestanding muscular sleeve, which forms part
Atrial septum
Ventricular septum
Sup.
Left
Right
AV separating structures
Inf.
Fig. 7.1 The four-chamber section through the heart, in

anatomical orientation, shows the atrial and ventricular septal
components, along with the location of the muscular structures
that separate the cavities of the right atrium and left ventricle. We
had previously considered the latter area to be an atrioventricular
(AV) muscular septum. We now recognise it to be a sandwich of
broadipose tissue, located between the layers of atrial and
ventricular musculature (see Figure 7.3). It is this area that is
decient in the setting of a common atrioventricular junction. Note
also that the superior rim of the oval fossa (arrow) is an infolding
between the right and left atrial walls.
152
Floor of oval fossa
Sup.
Left
Right
Inf.
Anteroinferior muscular buttress
Fig. 7.2 The heart has been sectioned in the four-chamber plane,
showing that the superior rim of the oval fossa is a deep
infolding (arrow) between the origin of the superior caval vein
from the right atrium (red star), and the entry of the right superior
pulmonary vein into the left atrium (white star). It is the oor of
the oval fossa, along with the anteroinferior muscular buttress,
which are the components of the atrial septum.
Tricuspid valve
Triangle of Koch
Apex
Inf.
Sup.
Base
Fig. 7.3 The surgical view, through a right atriotomy, shows the
Defect in oval fossa

Coronary sinus
of the supraventricular crest (Figure 7.7).

The small septal component interposing
between the ventricular outlets is
inextricably linked with the more extensive
component of the crest, the
landmarks of the triangle of Koch (triangle). This area is the atrial

aspect of the muscular atrioventricular sandwich. Note that this
patient also has a defect within the oval fossa.
ventriculoinfundibular fold, or inner heart

curvature (Figure 7.8). It is the muscular
wall separating the apical trabecular
components, therefore, which forms the
greater part of the muscular ventricular
septum. This part extends to the apex in

curvilinear fashion, reecting the
interrelationships of the banana-shaped
right ventricle and the conical left ventricle.
Reinforcing the right ventricular aspect of
153
Floor of oval fossa
Anteroinferior buttress
Sup.
Right
Left
Inf.
Adipose tissue
Fig. 7.4 This four-chamber section of a normal heart, taken across

the oor of the triangle of Koch, illustrates the differential
attachments of the atrioventricular valves (arrows). Note the
adipose tissue interposed between the right atrial wall and the
crest of the ventricular septum, which forms the meat in the
atrioventricular muscular sandwich.
Base
Post.
Ant.
Outlet component
Apex
Apical trabecular
component
Fig. 7.5 The dissection, seen in anatomical

orientation, shows how the ventricular septum
can be separated from the remainder of the
heart, and divided into brous and muscular
parts. The muscular part has been further
divided into segments corresponding to the
components of the right ventricle. Apart from
the apical part, however, these parts of the
right ventricle do not correlate with
comparable components on the left side of
the heart.
Inlet component
Membranous septum
this part of the septum is the septomarginal

trabeculation, or septal band. This muscular
strap has a body and limbs, the latter
extending to the base of the heart to clasp the
supraventricular crest. A series of

septoparietal trabeculations extend from its
anterocephalad surface and reach the
parietal ventricular wall. One of these, the
moderator band, is particularly prominent.

It crosses from the septomarginal
trabeculation to join the anterior papillary
muscle (Figure 7.9).
154
Left ventricular outlet
Base
Fig. 7.6 The section, simulating the oblique

Right
Left
Right ventricular inlet

Apex
subcostal echocardiographic cut, shows how

the inferoposterior part of the muscular
septum separates the right ventricular inlet
from the left ventricular outlet (double-headed
arrow).
Infundibular sleeve
Pulmonary trunk
Base
Post.
Ant.
Aortic root
Apex
Fig. 7.7 The dissection of the ventricular outow tracts, in

anatomical orientation, shows the free-standing sleeve of
infundibulum that supports the leaets of the pulmonary valve.
This is not a septal structure. Note the extensive tissue plane that
separates the infundibular sleeve from the aortic root (arrow).
155
Infundibular sleeve
Pulmonary valve
Aortic root
Sup.
Ant.
Post.
Inf.
Fig. 7.8 The dissection, seen in anatomical orientation, shows
how most of the supraventricular crest is formed by the

ventriculoinfundibular fold. Only a small part of crest, where it
inserts between the limbs of the septomarginal trabeculation
(star), can be removed so as to provide a communication with the
left ventricle. The area has no obvious anatomical boundaries.
Note that the distal part of the crest becomes continuous with the
free-standing muscular infundibular sleeve.
Septoparietal
trabeculations
Moderator band
Fig. 7.9 The septal surface of the right ventricle has been
Base
Ant.
Post.
Apex
Interatrial communications
There are several lesions that permit
interatrial shunting (Figure 7.10).
Although collectively termed atrial septal
defects, not all are within the connes of
displayed by making a window in the anterior wall to show the

septomarginal trabeculation, or septal band (black Y). The
supraventricular crest inserts between its basal limbs. The
moderator band takes origin from the apical part of its body,
crossing the cavity of the ventricle to become continuous with the
anterior papillary muscle. The band is but one of a series of
septoparietal trabeculations that arise from the anterior margin of
the major septomarginal trabeculation.
the normal atrial septum1. Only the

holes within the oor of the oval fossa2,
and the much more rare vestibular
defects found within the muscular
anteroinferior buttress3, are true
deciencies of the septal components

(Figure 7.2). The ostium primum
defect is the consequence of decient
atrioventricular septation. Its cardinal
feature is the commonality of the
156
Vestibular
defect
Atrioventricular
septal defect
Apex
Sup.
Inf.
Coronary
sinus defect
Base
Fig. 7.10 The cartoon shows the various holes
Superior sinus
venosus defect
Inferior sinus
venosus defect
Oval fossa defect
atrioventricular junction4. It will be

considered in our next section. Sinus
venosus defects, representing an
anomalous connection of a pulmonary
vein, which has retained its left atrial
connection5, are found at the mouths of
the caval veins58. The rare defect found
at the mouth of the coronary sinus is
the consequence of the disappearance of the
muscular walls that usually separate the
component of the coronary sinus running
through the left atrioventricular junction
from the cavity of the left atrium9.
Defects within the oval fossa are often
called secundum defects. Because they
represent persistence of the secondary
atrial foramen, rather than deciencies of
the secondary atrial septum, they should
properly be called ostium secundum
defects. We prefer to consider them as
defects within the oval fossa. They are, by
far, the most common type of interatrial
communication. They can be caused by
deciency (Figure 7.11), perforation
(Figure 7.12), or absence (Figure 7.13) of
the oor of the fossa. The oor is formed by
the ap valve of the oval foramen, itself
derived from the primary atrial septum.
When the haemodynamics of the shunt
across such a defect dictate surgical closure,
the hole is rarely likely to be small enough
to permit direct suture. Now, all but very

large defects within the oval fossa are likely
to be closed by the interventional
cardiologist. If attempts are made to close,
directly, defects large enough to justify
surgical intervention, the results may so
distort atrial anatomy as to result in
dehiscence. Irrespective of the size of the
septal deciency, it is always possible to
secure a patch to the margins of the oval
fossa. When placing sutures, the likeliest
potential danger relative to the rims is to
the artery supplying the sinus node
(Figure 7.14). This artery can course
intramyocardially through the anterior
margin of the oval fossa, or lie deep within
the superior interatrial fold. There is also a
remote chance of damaging the aorta when
placing stitches anteriorly, as this part of
the rim is related on its epicardial aspect to
the aortic root (Figures 7.14, 7.15). On
occasion, deciency of the posteroinferior
rims of the oval fossa permits holes within
the fossa to extend into the mouth of the
inferior caval vein (Figure 7.16). In these
circumstances, care must be taken not to
mistake a well-formed Eustachian valve for
the posteroinferior margin of the defect.
A patch attached to the Eustachian valve
would connect the inferior caval vein to the
left atrium. It is always prudent, therefore,
that permit interatrial shunting. Only the holes

in the oval fossa and the rare vestibular defects
are true deciencies of atrial septal structures.
to ensure continuity of the inferior caval

vein and right atrium following placement
of a patch used to close a deciency of the
oor of the oval fossa.
Sinus venosus defects are more rare
than defects within the oval fossa, and
present greater problems in repair. The
defect adjacent to the inferior caval vein
(Figure 7.17) is relatively rare5. It opens
into the mouth of the inferior caval vein
posterior to the connes of the oval fossa.
Usually the fossa itself is intact, but it can
be decient or probe patent. The essence of
the defect is an anomalous connection of
the right inferior pulmonary vein to the
inferior caval vein, the pulmonary vein
retaining its left atrial connection5. It is
much more frequent to nd sinus venosus
defects adjacent to the mouth of the
superior caval vein6,8. These defects, again,
are due to an anomalous connection of
one or more of the right pulmonary veins to
the superior caval vein, the pulmonary
veins retaining their left atrial connection6.
The defects are outside the connes of the
oval fossa, and hence are interatrial
communications rather than atrial septal
defects (Figure 7.18)1,2.
When sinus venosus defects are found
in relation to the superior caval vein, the
orice of the vein usually overrides the
157
Apex
Sup.
Inf.
Base
Fig. 7.11 The surgical view through a right atriotomy shows a

deciency in the ap valve of the oval fossa (star).
Apex
Sup.
Inf.
Base
Fig. 7.12 The surgical view through a right atriotomy shows three
perforations (arrows) in the ap valve of the oval fossa.
superior rim of the oval fossa (Figures 7.19,

7.20). More rarely, such defects can be
found when the caval vein is committed
exclusively to the right atrium
(Figure 7.21)6. All the defects are
associated with anomalous connections of

the right superior pulmonary veins, which
drain into the superior caval vein
(Figures 7.22, 7.23), often through more
than one orice (Figure 7.19), while
retaining their left atrial connection

(Figure 7.20). The difculty encountered
during surgical repair reects the need to
reconstruct the anatomy so as to reroute the
venous return and, at the same time, close
158
Apex
Inf.
Sup.
Fig. 7.13 The surgical view through a right atriotomy shows the
Base
absence of the entire ap valve of the oval fossa.
Sup.
Ant.
Post.
Inf.
Fig. 7.14 The dissection, made by transecting the atrial
Floor of oval fossa
Aortic root
the interatrial communication. This must

be done without obstructing venous ow
or, in the case of a superior defect,
damaging the sinus node. The sinus node is
related to the anterolateral quadrant of the
cavoatrial junction. It lays immediately
chambers, and illustrated in anatomical orientation, shows the

relationship of the artery supplying the sinus node to the
anterosuperior rim of the oval fossa. Note also the proximity of the
rim to the aortic root. The arrow shows the infolded anterior rim of
the fossa.
subepicardially within the terminal groove

(Figure 7.23). Its location should be
considered both when making the
atriotomy, and when placing sutures in the
atrial walls. Problems arise should it be
necessary either to suture in the area of the
node when rerouting the pulmonary

venous return, or if there is need to enlarge
the orice of the caval vein. The former risk
can be minimised with judicious supercial
placement of the sutures. The latter
problem is much greater. Because the
Infundibulum
Aortic root
Right atrium
Left atrium
Fig. 7.15 The computed tomogram shows the adjacency of the

anterosuperior margin of the oval fossa (yellow brace) and the
adjacent atrial walls to the aortic root.
Apex
Sup.
Inf.
Base

defect within the oval fossa (large star) extending into the
mouth of the inferior caval vein (arrow). Note the location of the
triangle of Koch (small star).
artery to the sinus node may pass either in

front of or behind the caval vein, the entire
cavoatrial junction is a potentially
dangerous area. Incisions across the
cavoatrial junction carry a high risk of
damaging the artery, or even the node
itself. Should it be deemed necessary to cut

across the junction, a much better option is
to perform the Warden operation10. This
involves detaching the superior caval vein,
and reattaching it to the excised tip of the
right atrial appendage11.
Another defect that permits interatrial

shunting, but which is outside the connes
of the true atrial septum, is part of a
constellation of lesions termed unroong of
the coronary sinus (Figure 7.24)9. In this
setting, a persistent left superior caval vein
159
160
Superior caval vein
Sup.
Ant.
Post.
Inf.
Intact oval fossa
Sinus venosus defect
Oval fossa
Coronary sinus
Apex
Sup.
Fig. 7.17 The specimen, viewed in anatomical orientation, shows

the features of an inferior sinus venosus defect. The margins of
the oval fossa are intact. The defect is due to an anomalous
connection of the middle and inferior right pulmonary veins to the
inferior caval vein. The anomalously connected veins retain their
left atrial connection.

Inf.
Pulmonary venous orifice
Base
usually drains directly to the left atrial roof

(Figure 7.25), entering the chamber between
the appendage and the left pulmonary veins.
Because of the unroong of the coronary
sinus into the cavity of the left atrium, the
mouth of the coronary sinus functions as an
defect at the mouth of the inferior caval vein. There is anomalous

drainage of the right inferior pulmonary vein, which retains its
left atrial connection. The oval fossa is intact. This is a typical
example of the inferior sinus venosus defect.
interatrial communication (Figure 7.26).

Evidence is frequently seen of the walls of
the coronary sinus and left atrium along the
anticipated course of the left superior caval
vein into and through the left
atrioventricular groove (Figure 7.24).
Sometimes the mouth of the coronary sinus

can seem to open normally to the right
atrium, but in the absence of its component
usually occupying the left atrioventricular
groove, and without persistence of a left
superior caval vein draining to the left atrial
161
Sup.
Ant.
Post.
Inf.
Overriding orifice of SCV
Intact oval fossa

a superior sinus venosus defect with overriding of the orice of
the superior caval vein (SCV). The probe (stars) has been passed
through the broadipose tissue occupying the intact superior
margin of the oval fossa. There is anomalous drainage of the two
right upper pulmonary veins, which have retained their left atrial
connection.
Superior caval vein
Right pulmonary vein
Fig. 7.20 The computed tomogram shows how, most frequently,
Atrial septum
Left atrium
roof. In this setting, the left ventricular

coronary veins drain directly into the cavity
of the left atrium. In these circumstances, the
mouth of the coronary sinus again functions
as an interatrial communication. This lesion
is the extreme form of the spectrum of
the mouth of the superior caval vein overrides the crest of the
atrial septum in the setting of a superior sinus venosus defect. Note
that the right pulmonary veins drain anomalously to the superior
caval vein while retaining their left atrial connection (white doubleheaded arrow).
fenestration of the walls of the coronary

sinus, providing communications with the
cavity of the left atrium (see Chapter 9).
Surgical treatment of interatrial
communications through the mouth of the
coronary sinus is dictated by the presence,
and connections, of the left superior caval

vein. If it is present, and in free
communication with the right superior
caval vein, or if there is no left-sided
superior caval vein, the mouth of the
coronary sinus can simply be closed. If the
162
Right upper pulm. veins
Sup.
Post.
Ant.
Inf.

a superior sinus venosus defect without overriding of the orice
of the superior caval vein. As in the specimen shown in Figure 7.18,
there is anomalous drainage of the right upper pulmonary
(pulm.) vein, which has retained its left atrial connection. Note the
distance between the defect and the oval fossa (double-headed
arrow). Note also the intact rims of the oval fossa.
Oval fossa
Apex
Intact oval fossa
Inf.
Sup.
Base
Fig. 7.22 This surgical view through a right atriotomy shows a

superior sinus venosus defect, recognised because it is outside the
connes of the intact oval fossa.
right atrial mouth of the sinus is to be

closed, decisions must be made concerning
the treatment of the left superior caval vein.
In the presence of an adequate venous
channel communicating with the
brachiocephalic vein, the left caval vein can
be ligated. If, in contrast, the left-sided
channel has no anastomoses with the right

side, consideration should be given to
construction of a left-sided cavopulmonary
anastomosis, the Glenn shunt.
Alternatively, a channel can be constructed
along the posteroinferior wall of the left
atrium, connecting the left atrial opening of
the left-sided vein with the mouth of the

coronary sinus.
Atrioventricular septal defects

It is becoming increasingly frequent for the
anomalies variously described as
Superior caval vein
Sinus node
163
Apex
Inf.
Sup.
Base
Fig. 7.23 This surgical view, through a median sternotomy of

Pericardium
the heart shown in Figure 7.20, illustrates the anomalous

connection of the right superior pulmonary vein. Note the site of
the sinus node lying in the terminal groove.
Base
Ant.
Post.
Apex
Fig. 7.24 The heart is shown from the left side in anatomical
Unroofed coronary sinus
endocardial cushion defects,

atrioventricular canal defects, or a
persistent atrioventricular canal, to be
described as atrioventricular septal
Mitral valve
orientation. There is unroong of the coronary sinus as it courses

through the left atrioventricular groove, draining a persistent
left superior caval vein, with ligreed remnants (stars) showing the
site of the walls that initially separated the vein from the left
atrium.
defects12. This is entirely apppropiate

because, in anatomical terms, the
malformations are due to not only the
absence of the membranous
atrioventricular septum, but also the

overlapping region of atrial and ventricular
musculatures that normally forms the oor
of the triangle of Koch. The structures are
164
Left atrial
appendage
Left atrium
Fig. 7.25 The computed tomogram shows a persistent left

superior caval vein draining to the roof of the left atrium, in the
absence of the walls that usually separate the course of the vein
through the left atrioventricular groove to the mouth of the
coronary sinus.
Sup.
Orifice of SCV
Ant.
Post.
Oval fossa
Inf.
Fig. 7.26 The right atrium from the heart illustrated in Figure 7.24
Eustachian valve
Interatrial communication
through mouth of coronary sinus
absent because the unifying feature of the

group is the commonality of the
atrioventricular junction (Figure 7.27)4.
The optimal title for the group, therefore,
would be atrioventricular septal defect with
common atrioventricular junction. This is
is shown. Because of the unroong of the walls of the persistent

left superior caval vein (SCV), the mouth of the coronary sinus
functions as an interatrial communication.
because, on rare occasions, defects of the

membranous atrioventricular septum can
be found in the setting of separate right and
left atrioventricular junctions. These
defects, rst described by Gerbode and
colleagues13, and often called Gerbode
defects, can take two forms. The more

frequent variant exists when shunting
across a ventricular septal defect enters the
right atrium through a decient tricuspid
valve (Figure 7.28). The more rare variant
is a true deciency of the atrioventricular
165
Sup.
Right
Left
Inf.
Right atrium
Left atrium
Fig. 7.27 The section of a specimen, cut in the four-chamber
Right ventricle
Left ventricle
orientation, shows a common atrioventricular junction (doubleheaded arrow) guarded by a common atrioventricular valve in a
heart with decient atrioventricular septation. The black brace
shows the atrioventricular septal defect, between the leading edge
of the atrial septum and the crest of the muscular ventricular
septum (stars).
Apex
Sup.
Inf.
Shunting through
perimembranous VSD
Base
Intact atrioventricular
membranous septum
Atrioventricular
conduction axis
component of the membranous septum

(Figures 7.297.32)14. The key to
differentiating the true defects from those
involving passage via a decient ventricular
Fig. 7.28 The cartoon shows how, when there is a deciency of

the septal leaet of the tricuspid valve, shunting across a
ventricular septal defect (VSD) can also produce left ventricular to
right atrial shunting. This is the so-called indirect Gerbode defect.
septum is to demonstrate the competence

of the tricuspid valve (Figure 7.32).
Although atrioventricular septal defects
do exist in the form of the Gerbode defect
in the setting of separate right and left

atrioventricular junctions, now it is usual to
presume the presence of a common
atrioventricular junction when considering
166
Aortic root
Left ventricle
Right atrium
Fig. 7.29 The section through the heart, replicating the
Sup.
Right
echocardiographic four-chamber cut, shows how the septal leaet

of the tricuspid valve divides the membranous septum into
interventricular and atrioventricular components. It is deciency of
the atrioventricular component (white double-headed arrow) that
underscores the existence of the direct Gerbode defect.
Left
Inf.
Apex
Sup.
Intact interventricular
membranous septum
Inf.
Base
Shunting through deficient

atrioventricular
membranous septum
Atrioventricular
conduction axis
decient atrioventricular septation. The

presence of the common junction
fundamentally distorts the overall anatomy
when compared to the normally separate
Fig. 7.30 The cartoon shows how a deciency of the

atrioventricular component of the membranous septum
produces the setting for direct shunting from the left ventricle to
right atrium. This is the direct Gerbode defect.
right and left atrioventricular junctions

(compare Figures 7.27 and 7.33).
Because of the lack of the membranous
and muscular atrioventricular structures,
there is no septal atrioventricular junction.

Instead, the leading edges of the atrial
septum and the ventricular septum, the
latter usually covered by the
167
Apex
Sup.
Inf.
Right atrium
Base
Tricuspid valve
Fig. 7.31 The image, taken in the operating room, shows a

deciency of the atrioventricular component of the membranous
septum.
Deficient atrioventricular membranous septum
Apex
Competent tricuspid valve
Inf.
Sup.
Base
Fig. 7.32 In the heart of the patient illustrated in Figure 7.31,

insufations of saline in the right ventricle reveals a competent
tricuspid valve, showing that the shunting from the left ventricle to
right atrium is across a deciency of the atrioventricular part of the
membranous septum; in other words, a direct Gerbode defect.
Shunting through deficient

atrioventricular membranous septum
atrioventricular valvar leaets, meet at the

superior and inferior margins of the
common atrioventricular junction
(Figure 7.34). These meeting points of the
septal structures typically divide the
common junction into more-or-less equal
right and left sides. An eccentric location of
the septal structures relative to the junction

produces ventricular imbalance, or a
double-outlet atrium.
It is the overall anatomy produced by
the common atrioventricular junction that
distorts the disposition of the
atrioventricular conduction axis. An
analogue of the triangle of Koch can be seen

within the leading edge of the atrial
septum15. Because of the common
atrioventricular junction, however, the
atrial septal myocardium makes contact
with the ventricular myocardium only
superiorly and inferiorly (Figure 7.34).
168
Sup.
Right
Left
Inf.
Fig. 7.33 This normal heart has also been sectioned in fourchamber orientation. It shows the right and left atrioventricular
junctions (double-headed arrows with red borders) guarded by
separate atrioventricular valves (compare with Figure 7.27). The
white double-headed arrow shows the separating atrioventricular
structures.
Atrioventricular septal defect
Conjoined bridging leaflets
Fig. 7.34 The specimen, seen in anatomical orientation, has been
Sup.
Ant.
Post.
Inf.
Normally formed atrial septum
Usually the atrioventricular node is

displaced inferiorly. It typically lies in a
nodal triangle, but not the triangle of Koch.
When seen by the surgeon, the nodal
triangle has the coronary sinus at its base,
with the atrial septum to the left-hand side,
and the attachment of the leaets of the
prepared to show the right side of the heart with decient

atrioventricular septation and separate right and left
atrioventricular orices, the lesion also known as an ostium primum
defect. It is the conjoined bridging leaets that produce the
separate valvar orices. The septal defect occupies the site of the
normal atrioventricular separating structures. Note that the atrial
septum itself is virtually normal, and the oval fossa is intact,
although the leading edge of the septum is bowed from the
atrioventricular junction in the margin of the septal defect. The
stars show how the meeting points of the septal structures divide
the common junction into its right and left sides.
effectively common atrioventricular valve

to the right-hand side (Figures 7.35, 7.36).
The atrioventricular conduction axis
penetrates the apex of the triangle. It then
courses on the crest of the muscular
ventricular septum, covered by the inferior
bridging leaet of the atrioventricular valve
(Figure 7.37). The proximity of the

coronary sinus to the apex of the nodal
triangle is pertinent to surgical closure of
the septal defect. Ideally, the surgeon will
place a patch so as to leave the coronary
sinus draining to the systemic venous
atrium. The best option is to deviate the
Apex
169
Inferior bridging leaflet

Inf.
Sup.
Base
Coronary sinus
Fig. 7.35 This view through a right atriotomy shows the surgeons
view of the atrial septal surface in a heart with an
atrioventricular septal defect and separate right and left
atrioventricular orices, or the ostium primum defect. Kochs
triangle is well formed (red star), but no longer contains the
atrioventricular node. The node is displaced posteroinferiorly to
the apex of a new nodal triangle (white star). The three dashed
lines show the options for placement of sutures so as to avoid
traumatising the atrioventricular conduction axis. The green line
runs directly from the inferior bridging leaet to the inferior
margin of the leading edge of the atrial septum. The yellow line
courses from the inferior bridging leaet to the right side of the
atrioventricular node and skirts the margins of the coronary sinus,
keeping the orice of the sinus to its right side. The blue line
courses to the right side of the atrioventricular node, but is placed
so as to leave the coronary sinus draining to the left atrium.
Fig. 7.36 In this heart, again shown as seen by the surgeon

Apex
Sup.
Leading edge of normally formed atrial septum
suture line towards the left-hand side of the

inferior bridging leaet at its junction with
the atrial septum, using supercial surgical
bites (green line in Figure 7.35)15. An
alternative is to stay on the right-hand side,
Inf.
Base
through a right atriotomy, there is less room around the coronary

sinus to place a patch so as to leave the sinus draining to the right
side (yellow dashed line). The site of the atrioventricular node is
shown again by the white star (compare with Figure 7.35). Note
that it is not within the well-formed triangle of Koch (red star). The
green and blue lines show the alternate options for placement of
the suture line, as indicated in Figure 7.35.
but place the suture line within the mouth

of the coronary sinus so as to reach the edge
of the atrial septum (yellow line in
Figure 7.35). The safety of this approach
depends on the margin between the
mouth of the coronary sinus and the

apex of the nodal triangle (compare
Figures 7.35 and 7.36). A further
alternative is to keep the suture line to the
right of the mouth of the coronary sinus,
170
Branching AV bundle
Analogue of triangle of Kochdoes not contain AV node
Atrioventricular (AV) node
Coronary sinus
Fig. 7.37 The cartoon shows the usual disposition of the

atrioventricular (AV) conduction axis in hearts with an
atrioventricular septal defect and common atrioventricular
junction. The variant with a common valvar orice is illustrated.
Atrial septum
Right mural leaflet
Bridging leaflets
Apex
Inf.
Sup.
Left mural leaflet
Base
placing the sinus to the left of the atrial

patch (blue lines in Figures 7.35 and 7.36).
Occasionally, the inferior portion of the
atrial septum itself can be decient. The
coronary sinus then opens more
posteriorly and medially through the left
Fig. 7.38 In this atrioventricular septal defect with separate right

and left valvar orices, the atrial septum is grossly decient. The
atrioventricular node is found at the point where the muscular
ventricular septum joins the inferior atrioventricular junction (star).
atrial wall. The conduction axis, however,

follows the course of the muscular
ventricular septum, with the node
formed at the site of its union with the
inferior atrioventricular junction
(Figure 7.38)16.
The feature that distinguishes between

the various forms of atrioventricular septal
defects is the morphology of the leaets of
the atrioventricular valve that guard the
common atrioventricular junction,
together with their relationship to the
Superior bridging leaflet
171
Sup.
Right
Left
Inf.
Fig. 7.39 This atrioventricular septal defect, positioned

Left mural leaflet
Right mural leaflet
septal structures bordering the defect17. In

any heart that lacks the separating
atrioventricular structures, the common
atrioventricular valve has ve leaets. The
arrangement is seen most readily when the
valve itself has a common orice
(Figure 7.39). Two of the leaets extend
across the ventricular septum, with their
tension apparatus attached in both
ventricles. These are the superior and
inferior bridging leaets. Two other leaets
are contained entirely within the right
ventricle. They are the anterosuperior and
inferior mural leaets. The fth leaet is
contained exclusively within the left
ventricle, and is also a mural leaet.
Although the two leaets found within the
right ventricle are comparable to similar
leaets of the tricuspid valve seen in the
normal heart, the left ventricular leaets of
the common atrioventricular valve
(Figure 7.40) bear no resemblance to a
normal mitral valve. In the normal mitral
valve, found with separate atrioventricular
junctions, the ends of the solitary zone of
apposition between the leaets, and the
papillary muscles supporting them, are
situated inferoanteriorly and
superoposteriorly within the left ventricle
(Figure 7.41). With this arrangement, the
anatomically and viewed from above, and with a common valvar

orice, has been dissected to show the arrangement of the ve
leaets that guard the common atrioventricular junction. The
white double-headed arrow shows the zone of apposition between
the two bridging leaets.
extensive mural leaet guards two-thirds of

the circumference of the valvar orice. The
left ventricular outow tract then
interposes between the aortic leaet of the
mitral valve and the ventricular septal
surface. In atrioventricular septal defects
with a common atrioventricular junction,
in contrast, the left ventricular papillary
muscles are deviated laterally, being
positioned superiorly and inferiorly12,17.
Because of this, the mural leaet is
relatively insignicant, and guards much
less than one-third of the circumference of
the left atrioventricular orice. The left
orice, in effect, is guarded by a valve
possessing three leaets, these being the
small mural leaet, and the more extensive
left ventricular components of the superior
and inferior bridging leaets (Figure 7.42).
It had originally been suggested by
Rastelli and colleagues18 that the common
valve possessed four rather than ve
leaets. They argued that the differing
morphology to be found in the setting of a
common valvar orice reected the
morphology of the anterior common leaet,
which is now usually described as the
superior bridging leaet. In the
arrangement they described as Type A,
they considered the anterior common
leaet to be divided, with the two

components both attached to the
ventricular septum. They considered their
Type B variant again to have a divided
anterior common leaet, but with both
parts attached to an anomalous papillary
muscle in the right ventricle. In their
Type C malformation, they interpreted the
arrangement in terms of an undivided
common anterior leaet, which was freeoating and attached in the right ventricle
to an apical papillary muscle. In reality, the
purported division of the anterior common
leaet is the site of coaptation between the
right ventricular part of the superior
bridging leaet and the anterosuperior
leaet of the right ventricle (Figure 7.43).
In the Rastelli Type A variant, the point of
coaptation is supported by the medial
papillary muscle of the right ventricle. The
variation noted by Rastelli and his
colleagues18 is then readily explained on
the basis of increased commitment of the
superior bridging leaet to the right
ventricle, with concomitant diminution in
size of the anterosuperior leaet of the right
ventricle (Figure 7.44). As the superior
bridging leaet becomes increasingly
committed to the right ventricle, its site of
coaptation with the anterosuperior leaet
172
Zone of apposition
Apex
Inf.
Sup.
Base
Left mural leaflet
moves towards the right ventricular apex

(Figures 7.45, 7.46)17. A further difference
between the hearts at either end of the
spectrum identied by Rastelli and
colleagues18 is that, with minimal bridging,
the superior bridging leaet is tethered by
cords to the crest of the ventricular septum
(Figure 7.43). With extreme commitment
of the supporting papillary muscle to the
right ventricle, in contrast, the superior
bridging leaet is always free-oating. The
variation noted by Rastelli and colleagues
reected the changing morphology of the
superior bridging leaet. There is also
variation in the arrangement of the inferior
bridging leaet. This does not, however,
reect its commitment to the two
ventricles, which is usually balanced. The
leaet is often divided along the inferior
edge of the ventricular septum, with the
attachments of the two components
providing a relatively clear zone of
separation. The edges are most frequently
attached to the septal crest, but there can be
a ventricular component to the defect
through intercordal spaces. Such potential
for shunting beneath the inferior bridging
leaet is almost always found when the
superior bridging leaet is free-oating.
It is the morphology of the bridging
leaets themselves that accounts for much
typical trifoliate formation of the left atrioventricular valve in a
heart with a decient atrioventricular septation and common
atrioventricular junction. It bears no resemblance to the formation
of the leaets as seen in the normal mitral valve. Note the extensive
zone of apposition between the two leaets that bridge the
ventricular septum (dashed white double-headed arrow).
of the remaining variability in

atrioventricular septal defects with a
common atrioventricular junction. The
overall valvar morphology is comparable in
all hearts with this specic phenotype. The
variability depends on the relationship of
the two bridging leaets to each other, or
their relationships, on the one hand, to the
lower edge of the atrial septum and, on the
other hand, to the crest of the muscular
ventricular septum. If these two features
are described separately, there is no need to
use terms such as complete, partial, and
intermediate when seeking to subdivide
the group. It is the use of these terms that,
in the past, has created most confusion in
description. From the surgical stance, if it
is possible to visualise a bare area at the
midportion of the ventricular septal crest;
this would constitute a complete lesion. If
the crest of the septum is covered by a
tongue of tissue that joins the bridging
leaets together, then the lesion is
considered either partial or intermediate.
The intermediate variant is characterised
by the presence of shunting at both atrial
and ventricular levels, but with separate
valvar orices within the common
atrioventricular junction.
The partial variant, with no ventricular
shunting, is also well described as the
ostium primum variant. The bridging

leaets are joined to each other along the
crest of the ventricular septum by a
connecting tongue of leaet tissue
(Figure 7.47). The essence of the ostium
primum defect, therefore, is the presence
of separate valvar orices for the right and
left ventricles within the common
atrioventricular junction. Because the heart
of necessity possesses a common
atrioventricular junction, the left valve
has three leaets, with an extensive
zone of apposition between the left
ventricular components of the superior
and inferior bridging leaets. This area, in
the past, was frequently described as a cleft.
It has no morphological similarity to the
cleft found in the aortic leaet of the
mitral valve in hearts with normal
atrioventricular septation and separate
right and left atrioventricular junctions19.
It is necessary surgically to close part, or all,
of this zone of apposition when repairing
ostium primum defects (Figure 7.48). The
resulting closure, nonetheless, does not
recreate a leaet comparable to the aortic
leaet of the normal mitral valve
(Figure 7.49).
The options for haemodynamic
shunting across the atrioventricular septal
defects, which largely colour the clinical
173
Aorta
Mitral valve
Fig. 7.41 This view of the left ventricle, taken from the apex with
the ventricular mass sectioned in its short axis, shows the
interrelationship of the normal mitral valve and the outow tract
to the aorta. Note the oblique position of the papillary muscles
supporting the solitary zone of apposition between the leaets of
the mitral valve, along with the extensive space between the aortic
leaet of the mitral valve and the septal surface of the left
ventricle (star).
Sup.
Left
Right
Inf.

Zone of apposition
Sup.
Left
Right
Inf.
Fig. 7.42 This view of the left atrioventricular valve and the
Left mural leaflet
outow tract in a heart with an atrioventricular septal defect is

taken in comparable fashion to the normal heart seen in
Figure 7.41, showing the short axis of the left ventricle. Note that
the left valve in the heart with decient atrioventricular septation
has three leaets, with papillary muscles positioned directly
superiorly and inferiorly, rather than being obliquely positioned as
in the normal heart. There is an extensive zone of apposition
between the bridging leaets (dashed white double-headed
arrow). Note also the way that the outow tract is squeezed
between the superior bridging leaet and the superior margin of
the left ventricle (star).
174
Inferior leaflet
Fig. 7.43 The image shows the location of the line of coaptation
Sup.
Ant.
Post.
Inf.
between the superior bridging and anterosuperior leaet of the

common atrioventricular valve as seen from the atrial aspect in an
atrioventricular septal defect with a common valvar orice (white
arrow). This arrangement, with minimal bridging of the superior
leaet, produces the so-called Rastelli Type A malformation. The
star shows the atrial component of the septal defect. Note that
there is the potential for multiple shunts through intercordal
spaces to occur at the ventricular level beneath the superior
bridging leaet. Note also the presence of anterosuperior and
inferior leaets in the right ventricle. The white arrow shows the
zone of apposition between the leftward margin of the
anterosuperior leaet and the right ventricular component of the
superior bridging leaet. It is a mistake to consider this
arrangement to represent a divided common anterior leaet.
Rastelli Type A
Sup.
Right
Left
Inf.
Bifid anterior muscle
Anomalous apical muscle
Anterior muscle
Rastelli Type B
Rastelli Type C
features, depend upon the relationship of

the bridging leaets to the septal
structures4. When there is a common
valvar orice, it is extremely rare for the
Fig. 7.44 The cartoon is shown as seen in

anatomical orientation and viewed from above
(compare with Figure 7.39). It shows the spectrum
of bridging of the superior leaet in hearts with
an atrioventricular septal defect and common
orice that underlies the classication introduced
by Rastelli and his colleagues18. As the superior
bridging leaet (pink) extends further into the
right ventricle, there is concomitant diminution in
size of the anterosuperior leaet (pink, crosshatched), and fusion of the anterior and medial
papillary muscles of the right ventricle, which
increasingly are attached towards the right
ventricular apex.
leaets to be attached directly to the crest of

the ventricular septum, although the extent
of their tethering by tendinous cords can
vary markedly. When the bridging leaets
lack direct attachments to the septal

structures, the potential exists for shunting
at both atrial and ventricular levels, the
magnitude of the shunts depending on the
175
Inferior leaflet
Fig. 7.45 The image is taken in the same orientation as in
Sup.
Post.
Ant.
Inf.
Figure 7.43. It shows how, in the Rastelli Type B variant of the

common valvar orice, the line of coaptation between the superior
bridging leaet and the anterosuperior leaet of the right ventricle
(white arrow) is displaced into the right ventricle. The papillary
muscle supporting the zone of coaptation has moved down the
ventricular septum towards the apex of the right ventricle. The
anterosuperior leaet itself is smaller than in the Rastelli Type A
variant, but there is also an inferior leaet in the right ventricle,
displaced upwards because of the opened right atrioventricular
junction. The star shows the atrioventricular septal defect.
Fig. 7.46 The image, taken to parallel the views seen in
Sup.
Ant.
Post.
Anomalous apical PM
Inferior leaflet
Inf.
prevailing haemodynamic conditions,

together with the extent of the tethering. If
the leaets are rmly attached to the
ventricular septum, shunting will be
Figures 7.43 and 7.45, but showing only the right ventricular
component of the superior bridging leaet, illustrates the Rastelli
Type C arrangement. Its line of coaptation with the anterosuperior
leaet of the right ventricle has moved even further into the right
ventricle (white arrow), with a concomitant decrease in size of the
anterosuperior leaet. The papillary muscle (PM) supporting the
line of coaptation is further towards the apex of the right ventricle.
The star shows the location of the muscular ventricular septum.(The
image is reproduced by kind permission of Mr. Bill Devine,
Childrens Hospital of Pittsburgh.)
conned at atrial level. This is the

arrangement found most frequently in the
typical ostium primum defect, with
separate right and left valvar orices within
the common junction, but with both

bridging leaets rmly fused to the crest of
the ventricular septum (Figure 7.50).
Much less frequently, the bridging leaets
176
Left mural leaflet
Fig. 7.47 This heart with an atrioventricular septal defect and
Sup.
Right
Left
Inf.
Right mural leaflet
separate right and left valvar orices is viewed in anatomical

orientation from above, after removal of the atrial chambers and
the arterial trunks (compare with Figure 7.39). The common
atrioventricular junction is guarded by a valve with separate
orices for the right and left ventricle. This is because a tongue of
leaet tissue (star) joins together the facing surfaces of the
bridging leaets (compare with Fig. 7.39). The white doubleheaded arrow shows the zone of apposition between the left
ventricular components of the bridging leaets.
Apex
Sup.
Inf.
Base
Left mural leaflet
can be rmly attached to the underside of

the atrial septum (Figure 7.51). This
arrangement will conne the potential for
shunting at the ventricular level. It
Fig. 7.48 This view, taken through a right atriotomy, shows the
trifoliate conguration of the left atrioventricular valve
subsequent to repair of the zone of apposition between the left
ventricular components of the bridging leaets (dashed black
double-headed arrow). Even after the surgical repair, the valve has
no similarity to the normal mitral valve (see Figure 7.49).
produces the true ventricular septal defect

of atrioventricular canal variety, as the
valve guarding the common
atrioventricular junction will have the
characteristics of a common
atrioventricular valve, rather than tricuspid
and mitral valves (Figures 7.517.55). The
potential also exists, nonetheless, for the
177
Aortic leaflet
Sup.
Right
Left
Fig. 7.49 This view through a right atriotomy shows a normal
Mural leaflet
Inf.
mitral valve. The structure bears no comparison to the trifoliate

valve shown in Figure 7.48.
Sup.
Left
Right
Inf.
leaets to be free-oating even in the

presence of separate valvar orices
(Figure 7.56). Most would consider the
lesion shown in Figure 7.56 as an
Left mural leaflet
Fig. 7.50 This heart with a common atrioventricular junction and

separate right and left valvar orices is shown from the left side in
anatomical orientation. The bridging leaets are rmly fused to
the crest of the ventricular septum (red dotted line), conning
shunting through the atrioventricular septal defect at the atrial
level. The white double-headed arrow shows the zone of
apposition between the left ventricular components of the
bridging leaets. This is the essence of the ostium primum defect.
intermediate defect. If the variability in

terms of attachment of the bridging leaets
is described, along with information
concerning the presence of a common
valvar orice or separate right and left

orices, there is no need to introduce the
concept of intermediate or transitional
variants. We recognise, nonetheless, the
178
Right atrium
Left atrium
Fig. 7.51 The four-chamber section is through an anatomical

Sup.
Left
Right
Inf.
Ventricular septal defect
specimen from a patient with decient atrioventricular septation

and a common atrioventricular junction (dashed white doubleheaded arrow). The inferior bridging leaet is rmly fused to the
undersurface of the atrial septum (star), conning shunting
through the atrioventricular septal defect at the ventricular level
(solid white double-headed arrow).
Oval
fossa
Sup.
Fig. 7.52 The specimen is viewed in anatomical orientation from

Right
Left
Inf.
value of the shorthand terms of partial,

intermediate, and complete variants,
providing that all working on the same
team understand the denitions used for
the different types.
the right side. The defect permits shunting at the ventricular level
only, because although the leaets of the atrioventricular valve
form a bridge across the crest of the ventricular septum into the left
ventricle, the atrial septum is intact (star).
Thus, in many patients with so-called

partial defects, with separate right and left
valvar orices within the common junction,
echo Doppler interrogation reveals the
potential for interventricular shunting
beneath the tongue that joins together the

bridging leaets, or otherwise through
intercordal spaces tethering the bridging
leaets themselves. Such patients are
well described as having separate valvar
179
Sup.
Left
Right
Inf.
Mural leaflet
Fig. 7.53 The image shows the left-sided aspect of another heart
with decient atrioventricular septation and a common
atrioventricular junction. This picture clearly shows the
attachments of the bridging leaets to the leading edge of the
atrial septum, conning shunting at the ventricular level. Note the
typical conguration of the left ventricular outow tract (star).
Apex
Inf.
Sup.
Base
Fig. 7.54 This picture, taken in the operating room, shows the
Intact atrial septum
orices, with predominantly atrial

shunting, but with the potential for
minimal ventricular shunting. The basic
morphology of the decient ventricular
septum, therefore, is comparable in all
patients having atrioventricular septal
right atrial aspect of an atrioventricular septal defect with a

common atrioventricular junction, in which shunting is conned at
the ventricular level because the bridging leaets are rmly
attached to the underside of the atrial septum. In this image, the
septal defect itself is not obvious.
defects with a common atrioventricular

junction12. As already emphasised, it is
this disposition that determines the
course of the ventricular conduction
pathways (Figure 7.34)13,20. Although the
hallmark of the malformation is the
common atrioventricular junction,

coupled with the absence of the
atrioventricular muscular and membranous
separating structures, there is also
hypoplasia of the muscular ventricular
septum to a greater or lesser extent. This
180
Apex
Inf.
Sup.
Base
Ventricular
septal defect
Fig. 7.55 This image shows how, when the surgeon separated
Intact atrial septum
Atrial component
Fig. 7.56 In this heart from a patient with an atrioventricular
Sup.
Ant.
Post.
Ventricular component
Inf.
involves disproportion between the inlet

and outlet dimensions of the ventricular
septum when compared to the normal heart
(compare Figures 7.57 and 7.58). The
degree of septal hypoplasia also varies with
the right ventricular components of the bridging leaets of the

heart shown in Figure 7.54, the common atrioventricular junction is
seen, with shunting at the ventricular level across the
atrioventricular septal defect.
septal defect (white double-headed arrow), with separate valvar

orices and shown in anatomical orientation, the bridging leaets
and the connecting tongue oat free of both atrial and ventricular
septal structures so that there are both atrial and ventricular
defects.
regard to the extent of scooping of the

septum (Figure 7.58). The scooping is
greater in hearts with a common valvar
orice than in those with separate right and
left orices. Although the scooping is
greater in hearts with a common valvar

orice, thus increasing the likelihood of
there being a ventricular component to the
defect, variability is still found among these
hearts. When there is minimal scooping, it
181
Sup.
Post.
Ant.
Inf.
Fig. 7.57 A normal heart is shown from the left side, the parietal
wall of the left ventricle having been removed. There is equality in
the inlet (blue double-headed arrow) and outlet (red doubleheaded arrow) dimensions of the ventricular septum, along with
the midseptal dimension (yellow double-headed arrow). (Compare
with Figure 7.58.)
Sup.
Post.
Ant.
Inf.
Fig. 7.58 The valvar leaets have been removed from the
ventricular mass in this heart with decient atrioventricular
septation and a common atrioventricular junction. The heart is
viewed from the left side and shown in anatomical orientation.
Because the leaets have been removed, there is no way of
knowing whether, originally, there was a common atrioventricular
orice, or separate valvar orices for the right and left ventricles.
Note the scooping of the ventricular septum (yellow doubleheaded arrow), and the disproportion between inlet (blue doubleheaded arrow) and outlet (red double-headed arrow) dimensions
of the ventricular mass.
is certainly possible to close the ventricular

component of the septal defect by attaching
the bridging leaets directly to the right
ventricular aspect of the ventricular
septum21. This manoeuvre, in fact, is
feasible in all patients with a common
valvar orice22,23, although not all are
convinced of its utility. Thus, the use of the

modied single patch approach remains
controversial. Some consider that the
technique creates potential narrowing of
the left ventricular outow tract. There
may be subtle technical differences that
produce the different results.
If the technique is used, care must be

taken not to damage the exposed
conduction tissues along the septal crest.
The non-branching bundle runs down the
crest of the scooped-out septum, and is
covered by the inferior bridging leaet.
The inferior leaet itself is often divided by
182
a midline raphe immediately above the

vulnerable non-branching bundle. The
branching component of the conduction
axis is found astride the midportion of
the septal crest. This is usually covered
by the connecting tongue and leaet
tissue in hearts with separate right and left
valvar orices. It is exposed in the

presence of a common orice and freeoating leaets. The right bundle branch
then runs towards the medial papillary
muscle. Anterior to this point, the septum
is devoid of conduction tissue
(Figures 7.59, 7.60)15.
Although not readily evident to the

surgeon during operation, the left ventricular
outow tract in atrioventricular septal defects
is intrinsically narrow24. It is much longer in
hearts with separate orices. This is because
of the attachment of the superior bridging
leaet to the ventricular septal crest (compare
Sup.
Ant.
Post.
Inf.
Right bundle branch
Atrioventricular conduction axis
Fig. 7.59 This heart came from a patient with an atrioventricular

septal defect with a common atrioventricular junction and
shunting conned at the atrial level; in other words, an ostium
primum defect. The disposition of the atrioventricular conduction
axis has been superimposed in red on the picture, as seen from the
right side.
Sup.
Post.
Ant.
Inf.
Fig. 7.60 The photograph shows the left ventricular aspect of the
Left bundle branch
Long non-branching bundle
heart illustrated in Figure 7.59, again with the location of the

atrioventricular conduction axis superimposed on the picture. Note
that the left ventricular outow tract (star) is distant from the
conduction tissues.
183
Sup.
Post.
Ant.
Inf.
Fig. 7.61 The heart, shown in anatomical orientation from the

left side, has been dissected to show the extent of the narrowed
outow tract in an atrioventricular septal defect with separate
right and left atrioventricular valves, and with the potential for
atrial shunting only. Because the superior bridging leaet is rmly
attached to the crest of the ventricular septum, the outow tract
has considerable length (black double-headed arrow).
Bridging leaflets
Sup.
Post.
Ant.
Inf.
Fig. 7.62 In this heart, again shown in anatomical orientation
Figures 7.61 and 7.62). The area is

prone to postoperative obstruction, and
surgical enlargement may be necessary.
Obstruction may be due to naturally
occurring lesions25, or to injudicious
placement of a prosthesis used to replace the
left atrioventricular valve. If a prosthesis
must be employed, the anatomy dictates
from the left side (compare with Figure 7.61), the outow tract
(black double-headed arrow) is much shorter in the presence of a
common atrioventricular valvar orice. The star shows the zone of
apposition between the bridging leaets.
insertion of a model with low prole,

otherwise resection of the shelf that exists
between the hingepoint of the superior
bridging leaet and the attachment of the
aortic valve24. The superior bridging leaet
can also be liberated from the septal crest,
inserting a gusset to enlarge the outow tract
(Figures 7.63 and 7.64).
Further variability is found in the

commitment of the common
atrioventricular junction to the
ventricular mass. Usually it is shared
equally, giving a balanced arrangement
(Figure 7.27). Should the common
junction favour one or other ventricle,
producing so-called right or left ventricular
184
Sup.
Left
Right
Inf.
Fig. 7.63 This heart has an atrioventricular septal defect with a

common atrioventricular orice, but with the superior bridging
leaet tethered across the subaortic outow tract (crossed black
arrows). It is photographed in anatomical orientation from the
left side.
Trifoliate left AV valve
Sup.
Left
Right
Inf.
Fig. 7.64 An incision has been made in the superior bridging

leaet of the heart shown in Figure 7.63, at the same time
detaching the leaet from the crest of the ventricular septum. The
incision makes it possible to insert a patch so as to widen the
outow tract (crossed black arrows).
Disattached superior bridging leaflet
dominance, the other ventricle is often

severely hypoplastic. This can have a major
inuence on the outcome of surgery, and
should always be assessed preoperatively.
In the setting of right ventricular

dominance, there is usually alignment
between the atrial and ventricular septal
structures at the crux. The essence of left
ventricular dominance, in contrast, is

malalignment between the atrial septum
and the muscular ventricular septum
(Figure 7.65). This produces an
Plane of
atrial septum
185
Sup.
Right
Left
Inf.
Left
ventricle
Cut-back coronary sinus
Bifid inferior bridging leaflet
Fig. 7.65 In this specimen from a patient with an atrioventricular

septal defect and common atrioventricular junction, shown in
anatomical orientation, there is gross malalignment between the
muscular ventricular septum (red dotted double-headed arrow)
and the atrial septum (black double-headed arrow). As a
consequence, the atrioventricular conduction axis originates from
an anomalous node (star) in the inferior aspect of the right
atrioventricular junction, rather than at the crux of the heart.
Malaligned muscular ventricular septum

Anomalous inferolateral node
Fig. 7.66 The cartoon shows how, when there is malalignment
Atrial septum comes to crux
between the atrial septum and the muscular ventricular septum,

with left ventricular dominance, the atrioventricular node is
formed at the point where the ventricular septum meets the
inferior atrioventricular junction.
arrangement analogous to straddling of the

tricuspid valve26. As with straddling the
tricuspid valve, this has major consequence
for the disposition of the atrioventricular
conduction axis27. Because of the septal
malalignment, the connecting
atrioventricular node is no longer to be
found at the crux. It continues to be formed
at the point where the malaligned muscular

ventricular septum meets the
This particular arrangement must be
identied preoperatively, as it can be
exceedingly difcult to recognise during
surgery. If unrecognised, it is likely that a
standard repair will damage the conduction
axis. Septal malalignment, therefore,

should be excluded in all cases of
atrioventricular septal defect with left
ventricular dominance. This arrangement
should also be distinguished from those
hearts in which the atrial septum is absent,
and the coronary sinus terminates in the
left atrium16.
186
Ventricular septal defects

When asked to close a clinically signicant
hole between the ventricles, the primary
concern of the surgeon is to ensure that the
task can be achieved in a safe and secure
fashion. The important anatomical
considerations reect the location of the
defect relative to the landmarks of the right
ventricle. These features determine the
proximity of the defect to the
atrioventricular conduction axis, and to the
leaets of the atrioventricular and arterial
valves. One categorisation of the defects28
was designed specically to focus the
attention of the surgeon on these pertinent
features. The essence of the system was
that, according to the anatomical features of
the margins of the defects as seen from the
morphologically right ventricle, all the
holes tted into one of three groups.
The rst group included all those holes
that, when viewed from the right ventricle,
had exclusively muscular borders
(Figure 7.67). The phenotypical feature of
the second group was that part of the right
ventricular border was composed of brous
continuity between the leaets of an
atrioventricular valve and an arterial valve
(Figure 7.68). The patients falling in the
third group were unied because part of

their right ventricular borders was made up
of brous continuity between the leaets of
the aortic and pulmonary valves
(Figure 7.69), with holes of this third type
showing additional variability depending
on whether the brous continuity extended
to include the leaet of an atrioventricular
valve. The defects in the patients making
up the third group, of necessity open
between the outow tracts of the ventricles.
Defects within the other groups, however,
need further description depending on
whether they open primarily to the inlet, to
the apical, or to the outlet components of
the right ventricle. There is thus an
additional feature that always requires
description, if present, namely
malalignment between the septal
components.
There are, of course, other
categorisations available for distinguishing
between types of holes between the
ventricles. One time-honoured system
identied four variants, and grouped them
in numerical fashion29. Another popular
system used developmental
considerations so as to distinguish between
the different holes30. We prefer the system
designed specically to emphasise the
surgical considerations (Figure 7.70).

When using this system, nonetheless, the
borders of the holes between the
ventricles must be assessed as seen by the
surgeon working through the
morphologically right ventricle
(Figures 7.677.69).
The essence of the largest group of
defects requiring surgical closure is that
part of the central brous body, specically
the area of brous continuity between the
leaets of the mitral, aortic, and tricuspid
valves, that forms a direct part of the rim of
the defect as seen from the right ventricle
(Figure 7.68). This brous area
incorporates the atrioventricular
component of the membranous septum,
which retains its integrity when ventricular
septation is incomplete, being an integral
part of the central brous body. The
defects, therefore, surround the
membranous part of the septum, and are
described, justiably, as being
perimembranous. In many instances, the
interventricular component of the
membranous septum is found as a fold of
brous tissue in the posteroinferior margin
of the defect (Figure 7.71).
The defects themselves represent the
unclosed embryonic interventricular
Apex
Inf.
Sup.
Base
Fig. 7.67 This view through a right atriotomy, and across the
orice of the tricuspid valve, shows a hole between the
ventricles that is enclosed by the walls of the muscular ventricular
septum (star).
187
Fibrous continuity
Fig. 7.68 This view, again through a right atriotomy with
Apex
Inf.
Sup.
Coronary
sinus
Base
Limbs of septomarginal
trabeculation
Pulmonary valve
Fig. 7.69 This surgical view, through a right ventriculotomy,
Base.
Right
Left
retraction of the leaets of the tricuspid valve, shows the brous

tissue of the atrioventricular septum forming part of the right
ventricular border, as viewed by the surgeon. This is the criterion
that permits the defect to be categorised as being
perimembranous.
Apex
Aortic valve
communication (Figure 7.72). They

presumably result from a deciency of the
muscular ventricular septum forming the
apical and cranial rims of the persisting
hole. Defects requiring surgical closure will
shows a hole between the ventricles that, at its cranial border, has
brous continuity between the leaets of the aortic and pulmonary
valves (black dotted line). The defect, opening into the ventricular
outlets between the limbs of the septomarginal trabeculation, is
doubly committed and directly juxta-arterial.
always be considerably larger than the area

occupied by the interventricular
membranous septum of the normal heart.
The degree of septal deciency has
important consequences for the disposition
of the axis of atrioventricular conduction

tissue31. In the normally formed heart, the
axis penetrates the atrioventricular
membranous septum to reach the crest of
the muscular septum. Having penetrated, it
188
Muscular defects
Perimembranous defects
Septomarginal
trabeculation
Juxta-arterial defects
Trabecular
Outlet
Doubly committed
juxta-arterial defect
Trabecular
Inlet
Inlet
Outlet
Central
fibrous
body
Triangle of Koch
and atrioventricular
node
Sinus node
Fig. 7.70 The cartoon, shown in surgical

orientation, illustrates the categorisation used
for differentiating the phenotypical variations
for holes between the ventricles. It combines
the phenotypical features shown in
Figures 7.67 to 7.69 with the location of the
hole relative to the components of the right
ventricle.
Remnant of
membranous
ventricular septum
Septal leaflet of
tricuspid valve
Apex
Inf.
Sup.
Fig. 7.71 In this heart, viewed through a right atriotomy and

through the orice of the tricuspid valve, a remnant of the
interventricular membranous septum is present in the
posteroinferior margin of the perimembranous defect.
Base
is sandwiched between the muscular

septum and the interventricular
component of the membranous septum
(Figure 7.73). In perimembranous defects,
when the atrioventricular connections are
concordant, so as to reach the crest of the
muscular ventricular septum, the axis

penetrates the area of continuity between
the leaets of the aortic and tricuspid valves
(Figure 7.74). When a remnant of the
interventricular membranous septum is
present, it lies immediately on top of the
atrioventricular bundle (Figure 7.74). If

such a remnant is seen at operation
(Figure 7.71), and is substantial, it may
safely be used for anchorage of sutures
placed supercially to anchor a surgical
patch.
189
Muscularising infundibulum
Developing aortic valve
Sup.
Left
Right
Inf.
Fig. 7.72 The image is a section taken from a dataset prepared
Developing tricuspid valve
Closing interventricular foramen
Aortic root
from a mouse embryo at embryonic day 13.5. It shows the margins

of the closing interventricular foramen (white brace), formed
dorsally by continuity between the cushions forming the leaets of
the aortic and atrioventricular valves. Perimembranous defects
reect failure to close this embryonic interventricular
communication.
Left
atrium
Atrioventricular
membranous
septum
Right
atrium
Left
ventricle
Sup.
Septal leaflet
of tricuspid valve
Left
Right
Right
ventricle
The location of the medial papillary

muscle, together with the apex of the
triangle of Koch, provides the guide for
predicting the location of the conduction
axis in almost all holes that are
Inf.
Fig. 7.73 This four-chamber section, shown in anatomical

orientation, reveals the position of the penetrating atrioventricular
bundle (star) in the normal heart. It is sandwiched between the
central brous body and the crest of the muscular ventricular
septum.
perimembranous, in other words the holes

bordered posteroinferiorly by brous
and tricuspid valves (Figure 7.75). The
only exceptions to this rule are the defects
associated with straddling and overriding

of the tricuspid valve27. The proximity of
the conduction tissues to the leaets of the
aortic and atrioventricular valves, however,
varies depending upon the precise area of
190
Aortic root
Septal leaflet
of tricuspid valve
Membranous flap
Fig. 7.74 This four-chamber section, again seen in anatomical

orientation, shows the position of the penetrating
atrioventricular bundle (star) in a heart with a perimembranous
ventricular septal defect (black brace). The black dotted line shows
the area of brous continuity between the leaets of the aortic
and tricuspid valves.
Turned back
septal leaflet
Fig. 7.75 This hole between the ventricles, shown in anatomical
Sup.
Left
Right
Membranous flap
Inf.
deciency of the muscular septum. It is

likely that all parts are decient to a certain
extent. It can usually be determined,
nonetheless, which part is most affected.
When a perimembranous defect extends to
orientation, is viewed from its right ventricular aspect. The

posteroinferior margin of the defect is made up of a brous
continuity between the leaets of the aortic and tricuspid valves
(black dotted line). In this setting, which makes the defect
perimembranous, the course of the conduction axis, shown by the
red dotted line originating from the triangle of Koch (white
triangle), is always positioned posteroinferiorly. Note that the
axis lies directly beneath a membranous ap. The septal leaet of
the tricuspid valve has been retracted.
open mostly into the inlet of the right

ventricle, its atrial margin, as viewed
through the tricuspid valve, is an area of
brous continuity between the leaets of
the aortic and tricuspid valves, but the
brous continuity also extends to involve

the leaets of the mitral valve (Figure 7.75).
The apex of the triangle of Koch is usually
deviated inferiorly towards the coronary
sinus. It is thus to the right-hand side of the
surgeon working through the atrium. The

axis of atrioventricular conduction tissue
penetrates this corner of the defect.
Usually, the non-branching and branching
bundles are carried on the left ventricular
aspect of the muscular septum as they
descend the right-hand margin of the
defect. The right bundle branch then
courses intramyocardially, surfacing
beneath the medial papillary muscle, which
is usually at the left-hand margin of the
defect. The non-coronary leaet of the
aortic valve is more to the left, and usually
distant from the rim of the defect, although
it often maintains brous continuity with
the septal leaet of the tricuspid valve
(Figure 7.76).
When perimembranous defects
requiring surgical closure are located so as
to open mostly towards the ventricular
apex, they are large. They typically open
additionally towards the inlet and outlet
components, and hence are conuent
defects. The triangle of Koch is not
deviated as far towards the coronary sinus
in such defects as when they open primarily
to the right ventricular inlet, but the righthand rim is still the major area at risk. The
medial papillary muscle tends to be at the
apex of such defects, and the non-coronary
leaet of the aortic valve is more closely
Tricuspid valve (through defect)
related to the atrial margin. The septal

leaet of the tricuspid valve is often cleft
or decient, an arrangement that may
permit shunting from the left ventricle to
right atrium, and hence producing an
indirect Gerbode defect (see
Figure 7.28)14. If the cleft in the septal
leaet of the tricuspid valve requires
surgical closure, it should be remembered
that the penetrating atrioventricular bundle
is located at its apex.
The third type of perimembranous
defect extends mostly so as to open into the
outlet of the right ventricle. The outlet
septum, along with the free-standing
subpulmonary infundibulum, can thus be
recognised separating the leaets of the
aortic and pulmonary valves, being
malaligned relative to the rest of the septum
(Figure 7.77). It is this type of defect that
others describe as being conoventricular30.
In the presence of anterocephalad
malalignment of the muscular outlet
septum, the aortic root overrides the crest
of the muscular ventricular septum
(Figures 7.78, 7.79). The medial papillary
muscle is on the right-hand margin of the
defect, and the axis of atrioventricular
conduction tissue is more distant from the
edge, being carried well down on the left
surface of the ventricular septum. The
Fig. 7.76 This illustration, seen in anatomical orientation, shows

Post.
Ant.
Inf.
non-coronary and right coronary leaets of

the aortic valve are much more closely
related to the left-hand margin
(Figure 7.79), and may prolapse towards
the right ventricle32.
The essential feature of muscular
defects is that, when viewed from the right
ventricle, they have exclusively muscular
borders (Figure 7.67). They can open into
the inlet, apical, or outlet parts of the right
ventricle. Always, nonetheless, ventricular
musculature will interpose between the
edges of the defect and the attachments of
the leaets of the valves. When a muscular
defect opens into the inlet of the right
ventricle (Figure 7.80), it is inferior to the
atrioventricular axis of conduction tissue.
When viewed by the surgeon through the
tricuspid valve (Figures 7.81, 7.82), the
conduction axis is located on the left-hand
margin of the defect. The proximity of the
axis to the edge depends upon the
adjacency of the defect to the intact
membranous septum. The basal margin of
the muscular septum, interposing between
the edge of the defect and the atrial septum,
separates the septal leaet of the tricuspid
valve from the mitral valve, preserving the
off-setting of the valvar hinges. Its size will
determine whether it is suitable to be an
anchorage for sutures.
Sup.
Membranous flap
191
the left ventricular aspect of the perimembranous defect that

opened primarily to the inlet of the right ventricle shown in
Figure 7.75. The black dotted line shows the brous continuity
between the non-coronary leaet of the aortic valve and the
tricuspid valve, via the membranous ap.
192
Malaligned outlet septum
Pulmonary trunk
Aorta
Fig. 7.77 This heart, shown in anatomical orientation, has been
Sup.
Left
Right
Inf.
Aortic-tricuspid valvar continuity
sectioned to replicate the subcostal oblique long axis

echocardiographic projection. The perimembranous defect (star)
opens towards the outlet of the right ventricle. The muscular outlet
septum is now recognised in its own right. It is malaligned
relative to the muscular ventricular septum, and attached
anterocephalad relative to the septomarginal trabeculation.
Overriding aortic valve
Sup.
Left
Right
Perimembranous defect
Inf.
Defects opening through the apical part

of the septum can be single (Figure 7.83),
double, or multiple (Figure 7.84). They are
unrelated to the proximal parts of the
Fig. 7.78 This is the heart shown in Figure 7.77 prior to making
the section to correlate with the echocardiographic plane. The
ventricular septal defect opens into the outlet of the right
ventricle, with the aortic valve overriding the crest of the
ventricular septum.
conduction tissue axis, but may be related

to ramications of the distal bundle
branches. The right ventricular aspect of
such defects is frequently obscured by the
coarse apical trabeculations. Multiple

defects, if small, may not be visible through
a right ventriculotomy. The septal
deciencies are much more readily
193
Apex
Inf.
Sup.
Base
Fig. 7.79 This view is through a right atriotomy, subsequent to

Incised leaflet
of tricuspid valve
Aortic valvar leaflet
making an incision at the junction of the septal and anterosuperior

leaets of the tricuspid valve. It shows a perimembranous
defect opening into the outlet of the right ventricle. The leaets of
the aortic valve are overriding the ventricular septal crest to the
left-hand margin of the defect.
Tricuspid
valve
Sup.
Left
Right
Inf.
identied from the left ventricular aspect.

Inspection from the left side will often
demonstrate that the defect is a solitary
hole (Figure 7.85). The multiple right
Fig. 7.80 This heart, photographed from the right side in

anatomical orientation, has a muscular ventricular septal defect
opening into the inlet of the right ventricle. The atrioventricular
conduction axis (black dotted line) runs anterocephalad relative to
the defect. In this instance, it is well removed from the superior
margin of the hole (star).
ventricular openings (Figure 7.84) simply

reect crossing of the solitary defect by the
right ventricular apical trabeculations33.
Multiple small defects, nonetheless, can
involve the entirety of the apical ventricular

septum. This produces the swiss-cheese
septum, an arrangement that can be
notoriously difcult to close surgically.
194

Apex
Inf.
Sup.
Base
Fig. 7.81 This surgical view shows a muscular defect opening into
Inlet muscular defect
the inlet of the right ventricle, viewed through the orice of the
tricuspid valve. The conduction axis (black dotted line), arising
from the apex of the triangle of Koch (cross-hatched triangle), is
seen to the left-hand side of the surgeon working through the
tricuspid valve.
Apex
Inf.
Sup.
Base
Fig. 7.82 This view of the heart shown in Figure 7.81, again seen
through a right atriotomy and the orice of the tricuspid valve,
shows the musculature of the ventricular septum interposing
between the basal margin of the defect (star) and the hinge of the
septal leaet of the tricuspid valve. The triangle of Koch (crosshatched triangle) is to the left-hand side of the surgeon relative to
the defect.
Basal muscular rim
Muscular defects opening into the

outlet of the right ventricle are relatively
rare in patients with concordant
connections. If small, the endocardium
may appear to be heaped up at the edges to
produce a brous rim (Figure 7.86). Larger
defects opening into the right ventricular

outlet typically show malalignment of the
muscular outlet septum (Figure 7.87).
Close inspection will show whether the
posterocaudal limb of the septomarginal
trabeculation is fused with the
ventriculoinfundibular fold to form a
muscular posteroinferior rim to the defect,

thus distinguishing the hole from a
perimembranous defect with outlet
extension. When present, the fusion of
these muscle bars separates the edge of the
defect from the axis of atrioventricular
conduction tissue. The superior rim is
195
Apex
Inf.
Sup.
Base
Fig. 7.83 This defect is seen through a right atriotomy with the
tricuspid valve retracted. It shows a muscular defect (star) opening
into the outlet of the right ventricle.
Pulmonary infundibulum
Tricuspid
valve
Sup.
Fig. 7.84 In this heart, viewed in anatomical orientation, it

Left
Right
seems that there are three muscular defects (arrows) opening

anteriorly into the apical trabecular component of the right
ventricle. Note, however, the presence of the septoparietal
trabeculations (stars).
Inf.
the muscular outlet septum, combined with

the free-standing subpulmonary
infundibulum. These tissues separate the
leaets of the pulmonary valve from the
right coronary leaet of the aortic valve, the
latter attached to its left ventricular

surface. If this superior muscular rim is
attenuated, the leaet of the aortic valve
may again prolapse through the defect
(Figure 7.87).
The third type of ventricular septal

defect is the one that is doubly committed
and juxta-arterial. The phenotypical
feature of this defect is absence of both the
muscular outlet septum, and the posterior
196
Aorta
Sup.
Post.
Ant.
Inf.
Left ventricle
Fig. 7.85 The left ventricular view, seen in anatomical

orientation, shows the left ventricular aspect of the heart
illustrated in Figure 7.83. In reality, there is a solitary defect (star) in
the muscular septum but it is crossed by the septoparietal
trabeculations on the right side, giving the spurious impression of
multiple defects.
Pulmonary valve
Sup.
Tricuspid valve
Left
Right
Inf.
aspect of the free-standing subpulmonary

infundibulum. Because of the absence of
these structures, the facing leaets of the
aortic and pulmonary valves are in brous
Fig. 7.86 The heart is photographed in anatomical orientation,

showing a muscular defect (star) opening into the outlet of the
right ventricle. Note the accretion of brous tissue at the edges of
the defect, reducing its size.
continuity, producing a brous raphe that

forms the superior rim of the defect
(Figures 7.69, 7.88). The leaets can be
attached at the same level, albeit that in
some hearts part of the aortic sinus may

interpose between them, producing valvar
off-setting. In either event, sutures can be
secured in the region of brous continuity.
197
Prolapse of aortic leaflet
Pulmonary valve
Sup.
Left
Right
Muscular outlet defect
Inf.
Fig. 7.87 In this heart, viewed in anatomical orientation, the right

coronary leaet of the aortic valve prolapses minimally through a
muscular defect that opens into the outlet of the right ventricle.
Muscular posteroinferior rim
Fig. 7.88 In this heart, the defect opening into the outlet of the
Sup.
right ventricle (star) is doubly committed and juxta-arterial, due to

failure of muscularisation of the subpulmonary infundibulum.
The roof of the defect is made up of a brous continuity between
the leaets of the aortic and pulmonary valves (black dotted
line). Note the extensive muscular posteroinferior rim to the defect
that protects the atrioventricular conduction axis.
Left
Right
Inf.
The doubly committed defect can also be

found with overriding of the orice of the
aortic valve (Figure 7.89). In most
instances, the inferior rim of the defect is
similar to that found in the muscular defect

that opens into the outlet part of the right
ventricle, the posterocaudal limb of the
septomarginal trabeculation fusing with the
ventriculoinfundibular fold (Figure 7.88).

When present, this muscular rim separates
the axis of atrioventricular conduction
tissue from the edge of the defect.
198
Overriding of
aortic valve
Fig. 7.89 This heart, photographed in anatomical orientation,

Sup.
Left
Right
Inf.
also has a doubly committed and juxta-arterial ventricular septal

defect opening into the outlet of the right ventricle in the absence
of muscularisation of the muscular subpulmonary infundibulum.
There is brous continuity between the leaets of the arterial
valves (blue dotted line). Note the extensive overriding of the
orice of the aortic valve.
Aortic-pulmonary fibrous continuity
Sup.
Left
Right
Inf.
Fig. 7.90 This doubly committed and juxta-arterial ventricular
Fibrous continuity with tricuspid valve
Occasionally, the muscular bundles do not

fuse. The defect then extends to be
bordered by brous continuity between the
leaets of the aortic and tricuspid valves,
making it perimembranous as well as
septal defect (star), shown in anatomical orientation, extends

so that its posteroinferior margin is formed by a brous continuity
between the leaets of the aortic and tricuspid valves, making
it additionally perimembranous. The pathognomonic feature is the
valves.
doubly committed (Figure 7.90). The

conduction axis, therefore, is much closer
to its inferior corner. It is the doubly
committed variant that is particularly prone
to set the scene for prolapse of the leaets of
the aortic valve32. Such defects are also

much more frequent in oriental as opposed
to occidental populations34.
Although the descriptions thus far have
related to ventricular septal defects in
patients having concordant atrioventricular

and ventriculoarterial connections, this
topology, and the guidance it gives to the
site of the conduction axis, is equally valid
for patients with concordant
atrioventricular connections, but abnormal
ventriculoarterial connections (see
Chapter 8). The only exception to the rules
we have described for recognition of the
location of the atrioventricular conduction
axis is produced by overriding and
straddling of the tricuspid valve (see later).
This arrangement results in a particular
type of defect that opens into the inlet of
the right ventricle. As we have described,
defects with markedly different
phenotypical features, and with markedly
different dispositions of the
atrioventricular conduction axis, can open
towards the inlet of the morphologically
right ventricle. This is why it is insufcient
simply to describe them as inlet defects35.
The commonest defects opening into the
right ventricular inlet are probably the ones
that are perimembranous (Figure 7.74).
These, and the ones associated with
straddling and overriding of the tricuspid
valve, have been described as being of the
atrioventricular canal type. It is certainly
the case that hearts with a common
atrioventricular junction can exist with

shunting exclusively at the ventricular level
(Figure 7.52). Patients with this
morphology, however, need to be
distinguished from those with either
perimembranous defects opening into the
right ventricular inlet, or those associated
with straddling and overriding of the
tricuspid valve, as both the latter variants
are found in the setting of separate
atrioventricular junctions. All of these
variants must be distinguished from
muscular defects opening into the inlet of
the right ventricle (Figure 7.80). There is
similar phenotypical variability among
defects opening into the right ventricular
outlet, but the major feature of surgical
signicance in this latter setting is to
determine whether a muscular bar is
interposed between the caudal rim of the
defect and the atrioventricular conduction
axis (compare Figures 7.88 and 7.90).
MALFORMATIONS OF THE
ATRIOVENTRICULAR VALVES
The pathological lesions that affect
atrioventricular valves, both acquired and
congenital, are legion. Not all are
199
amenable to surgical repair. We will

concentrate on features of immediate
surgical relevance.
The anatomy of atrioventricular valves
themselves indicates that problems may be
encountered at the atrioventricular
junction, which is contiguous with the
valvar annulus, in the leaets, or in
the tension apparatus. Sometimes all the
components of the valve, along with the
entire atrioventricular connection, are
totally absent. This produces the
commonest variant of atrioventricular
valvar atresia, which is discussed in
Chapter 8. The lesions to be considered in
this section can involve either the
morphologically tricuspid or mitral valves.
Because the tricuspid valve usually
functions in an environment of low
pressure, the lesions are more frequently
manifest when affecting the mitral valve.
We will deal with the respective lesions in
turn, indicating their proclivity towards
one or the other valve.
Of considerable surgical signicance is
overriding of the atrioventricular
junction. This means that the valvar
orice is looking into both ventricles,
positioned astride a septal defect
(Figure 7.91)27. Almost always, this is
Left atrium
Right atrium
Fig. 7.91 This heart has been sectioned to replicate the four-
Sup.
Left
Right
Inf.
chamber echocardiographic view, and is shown in anatomical

orientation. There is overriding of the orice of the tricuspid valve
(white double-headed arrow), and straddling of its tension
apparatus (white arrows). Note the gross malalignment between
the atrial and ventricular septal structures, the star marking the
crest of the muscular ventricular septum.
200
associated with straddling of the valvar

tension apparatus, with the tendinous
cords attached to both sides of the
muscular ventricular septum
(Figure 7.91)27. Although it is the mode
of insertion of the tension apparatus
across the septum that determines the
surgical options, the degree of override is
also important. Overriding of the
tricuspid valve typically is associated
with malalignment between the atrial
and ventricular septums, and this has
major consequences in terms of
arrangement of the conduction tissues.
Straddling and overriding, nonetheless,
can affect either valve, and can occur in
various segmental combinations27.
Straddling in the setting of a double-inlet
ventricle, and with discordant
atrioventricular connections, is
considered in Chapter 8. Here, the
concern is with straddling valves
coexisting with concordant
When the mitral valve straddles, it does
so through an interventricular
communication that opens into the outlet
of the right ventricle (Figure 7.92)27. This
arrangement does not produce

atrioventricular septal malalignment, so
that the muscular ventricular septum is
normally related to the atrial septum at the
crux, and the atrioventricular conduction
axis is normally disposed. It is the superior
papillary muscle of the mitral valve that is
abnormally attached within the right
ventricle. It typically arises from the
septomarginal trabeculation alongside, but
separate from, the anterior papillary muscle
of the tricuspid valve. This arrangement,
seen with either discordant
ventriculoarterial connections, or a doubleoutlet right ventricle with a subpulmonary
defect, producing the TaussigBing
malformation, can seriously compromise
surgical repair. We discuss the problems in
greater detail in Chapter 8.
Straddling of the tricuspid valve is
found when the ventricular septal defect
opens into the inlet of the right ventricle
(Figure 7.93)27. It can exist as an isolated
lesion, or be found with tetralogy of
Fallot, or other abnormal
ventriculoarterial connections such as
transposition. The phenotypical feature is
that the muscular ventricular septum is
malaligned relative to the atrial septum,

no longer inserting at the crux
(Figure 7.91). The conduction axis,
which is carried on the muscular
ventricular septum, originates from an
anomalous node in the inferolateral
margin of the right atrioventricular
junction27, found at the point where the
muscular septum comes into contact
with the junction (Figures 7.94, 7.95).
There is a rudimentary node found within
the regular triangle of Koch, but it makes
no contact with the ventricular
musculature. The septal leaet of the
tricuspid valve is usually tethered to the
enlarged inferoseptal papillary muscle of
the mitral valve (Figure 7.96). A
miniseptation procedure is often
necessary for complete ventricular
repair, which carries a high risk of
producing heart block36. It is possible,
nonetheless, to place a patch by sewing
the stitches exclusively in the straddling
leaet of the tricuspid valve (Figures 7.97,
7.98). This ensures avoidance of the
conduction tissues. An alternative option
is to convert the patient to the Fontan
circulation.
Straddling mitral valve

Sup.
Left
Right
Inf.
Tricuspid valve
Fig. 7.92 This heart has concordant atrioventricular and

discordant ventriculoarterial connections. It is viewed in anatomical
orientation, showing straddling of the tension apparatus of the
mitral valve through a defect opening into the outlet of the right
ventricle.
201
Fig. 7.93 The heart is photographed in anatomical orientation. It

shows the right atrial aspect of a straddling and overriding tricuspid
valve in the setting of concordant atrioventricular connections.
There is gross malalignment between the muscular ventricular
septum, which carries the atrioventricular conduction axis (black
dotted line), and the atrial septum. Because of this, the
connecting atrioventricular node is formed inferiorly in the
atrioventricular junction (star), and is not in the regular triangle of
Koch (triangle).
Sup.
Left
Right
Inf.
Apex
Inf.
Sup.
Base
Fig. 7.94 The image shows an operative picture through a right

atriotomy in a patient with concordant atrioventricular
connections complicated by straddling and overriding of the
tricuspid valve with basically concordant atrioventricular
connections. Note the malalignment of the muscular ventricular
septum, which carries the atrioventricular conduction axis (white
dotted line). The axis arises from an anomalous posteroinferior
atrioventricular node (star).
Straddling tension apparatus
Dilation of the atrioventricular

junction occurs almost exclusively as an
acquired lesion. A dilated mitral valvar
orice is most frequently secondary to
myocarditis. When surgical narrowing of

the orice is indicated, it can be
accomplished using various annuloplasty
techniques, without resorting to
replacement of the valve. Dilation of the

tricuspid valvar orice is seen most
frequently as a result of right heart
failure.
202
Malaligned ventricular septum
Apex
Fig. 7.95 The cartoon shows the anomalous location of the

atrioventricular conduction axis, as seen by the surgeon operating
through a right atriotomy in the setting of straddling and
overriding of the tricuspid valve. The rudimentary node found at
the apex of the regular triangle of Koch does not make contact
with the ventricular musculature.
Inf.
Sup.
Base
Aorta
Mitral valve
Sup.
Post.
Ant.
Straddling tricuspid valve
Inf.
More of a challenge surgically is the

dilation that accompanies Ebsteins
malformation37. The crucial feature of this
anomaly is the attachment of the hingepoint
of the septal and mural leaets of the
tricuspid valve towards the junction of the
Fig. 7.96 This picture, taken in anatomical orientation, shows

the left ventricular aspect of the heart shown in Figure 7.93. Note
the straddling part of the tension apparatus supporting the
tricuspid valve.
inlet and apical trabecular components of

the right ventricle, rather than at the
atrioventricular junction (Figure 7.99)38.
The displacement is usually described as
being downwards. In reality, there is
rotational displacement of the valvar orice
around the area of the central brous body.

The anterosuperior leaet is less affected in
terms of its junctional attachment, but shows
important variations in its distal
attachments39. These can be focal
(Figure 7.100). In more severe cases, the
203
Sup.
Post.
Ant.
Inf.
Mitral valve
Fig. 7.97 In this heart, from a patient with straddling and
Sutures securing patch
overriding of the tricuspid valve, and shown here from the left
ventricle in anatomical orientation, the surgeon placed a patch to
close the malalignment of the ventricular septal defect, keeping his
sutures in the leaets of the overriding valve, and avoiding the
abnormally located atrioventricular conduction axis (white dotted
line [axis] and cross-hatched area [node] superimposed on the
photograph). (Compare with Figure 7.96.)
Sup.
Right
Left
Inf.
Fig. 7.98 The image shows the right atrial aspect of the heart
seen also in Figure 7.97. It is photographed in anatomical
orientation, demonstrating how the surgeon was able to close
the ventricular septal defect successfully, at the same time avoiding
the atrioventricular conduction axis (black dotted line). The site of
the abnormal atrioventricular node is shown by the star.
leading edge of the leaet is attached in

linear fashion, severely restricting antegrade
ow into the pulmonary trunk
(Figure 7.101). In the most severe form, the
anterosuperior leaet completely blocks this
junction, producing tricuspid atresia in the
setting of Ebsteins malformation (see also

Chapter 8).
Ebsteins malformation requires surgical
treatment when there is signicant dilation
of the true atrioventricular junction, and
when the wall of the inlet component of the
right ventricle is both dilated and thinned.

Reparative operations often require
placement of sutures in the area of thinning.
Particular care should be taken to avoid the
right coronary artery and its branches. In the
septal area, the triangle of Koch remains
204
Septal leaflet
Anterosuperior
leaflet
Atrialised
inlet
Fig. 7.99 The heart is photographed from the right side in

Apex
Inf.
Sup.
Base
anatomical orientation. The hinge line of the septal leaet of the

tricuspid valve has been displaced rotationally (red dotted line)
away from the atrioventricular junction, shown by the black dotted
line. This feature, incorrectly described as downwards
displacement, is the hallmark of Ebsteins malformation. Note also
the marked dysplasia of the septal leaet. The black doubleheaded arrow shows the thinned wall of the atrialised component
of the right ventricular inlet.
Sup.
Left
Right
Inf.
Fig. 7.100 This picture, photographed in anatomical orientation
Inferior leaflet
Septal leaflet
the guide to the atrioventricular conduction

axis (Figure 7.102). Ebsteins malformation
involving the left-sided morphologically
tricuspid valve when the atrioventricular
from the right ventricle, shows the ventricular aspect of the heart
demonstrated in Figure 7.99. The anterosuperior leaet of the
abnormal tricuspid valve is tethered in focal fashion, with normal
attachments to the medial and anterior papillary muscles. The
rotational displacement has produced a bifoliate valve hinged at
the junction of the inlet and apical trabecular components of the
right ventricle.
connections are discordant is discussed

in Chapter 8. Rarely, the normally
located morphologically mitral valve can
show Ebsteins malformation. It is the hinge
of the mural leaet that is displaced away

from the atrioventricular junction40,41.
Malformations of the leaets can be
summarised in terms of dysplasia, prolapse,
205
Sup.
Left
Right
Linear attachment
Septal leaflet
Inf.
Fig. 7.101 This heart also has Ebsteins malformation, and is

photographed in anatomical orientation to show the ventricular
aspect of the abnormal tricuspid valve. In this example, the
anterosuperior leaet has grossly abnormal linear attachments
towards the apex of the right ventricle. The valvar orice is a mere
keyhole (white double-headed arrow), opening directly into the
subpulmonary infundibulum.
Sup.
Ant.
Post.
Inf.
Atrialised
inlet
Inferior leaflet
and clefting. Dysplastic valves show

thickening and heaping up of the substance
of the leaets, usually with obliteration of the
intercordal spaces. A dysplastic valve may
pose a signicant surgical problem. It is seen
frequently with atresia of the outow tract,
and is an integral part of Ebsteins
Fig. 7.102 This heart with Ebsteins malformation, viewed in

anatomical orientation as seen from the right atrium, shows
how the triangle of Koch (cross-hatched triangle) remains the
guide to the atrial components of the atrioventricular conduction
tissue axis, despite the abnormal attachments of the septal
leaet. Note again the rotational displacement of the hinge of the
septal leaet (red dotted line) relative to the atrioventricular
junction (black dotted line).
malformation42. Isolated dysplasia

(Figure 7.103) is exceedingly rare except in
neonatal life43, when it is often a fatal lesion.
Prolapse occurs more frequently, and
usually involves the mitral valve
(Figure 7.104). It is usually associated
with deciency of the tension apparatus44,
particularly elongation of the tendinous

cords (Figure 7.105). It may be sufciently
severe to warrant valvar replacement, but the
prolapsing leaets can be repaired by various
techniques, including cordal shortening
(Figure 7.106), and insertion of annular rings
(Figure 7.107).
206
Sup.
Left
Right
Septal leaflet
Inf.
Fig. 7.103 The right ventricle in this heart is opened in

Inferior leaflet
clam-shell-like fashion and photographed from the ventricular

apex. The tricuspid valve, although grossly dysplastic, has normal
junctional attachments (black dotted lines). This is not Ebsteins
malformation.
Prolapsing aortic leaflet
Sup.
Right
Left
Fig. 7.104 This surgical view of the mitral valve, taken

Inf.
through the dome of the left atrium, shows prolapse of the aortic
leaet.
The true cleft of the aortic leaet of the

mitral valve can be repaired simply by
reconstituting its edges. Such an isolated
cleft of the aortic leaet (Figure 7.108) has
to be distinguished from the lesion often
described as a cleft in the setting of
atrioventricular septal defects19. The latter

entity (Figure 7.109) is the zone of
apposition between the left ventricular
components of the bridging leaets of the
common atrioventricular valve. Along with
the short mural leaet, the bridging leaets
guard the left side of the common

atrioventricular junction in trifoliate
fashion.
We have already discussed some of the
abnormalities of the tension apparatus that
accompany malformations of the junction
207
Sup.
Post.
Ant.
Inf.
Fig. 7.105 In this specimen, photographed in anatomical

orientation, there is gross elongation of the cords supporting the
middle scallop of the mural leaet of the mitral valve (arrow),
which is prolapsed (star).
Sup.
Right
Left
Inf.
or leaets, such as straddling tension

apparatus. Of those which remain, the
so-called parachute deformity is probably
the most worrisome lesion, apart from rare
Fig. 7.106 As shown in Fig. 7.105, when the leaets of the mitral
valve are prolapsed, the cords supporting them are usually
elongated. This view shows how the surgeon has shortened the
elongated cords by incising the papillary muscle and suturing the
cords within the muscle (arrow).
anomalies such as arcade lesions45. Some

confusion exists about the denition of a
parachute valve. It is considered logically as
fusion of the papillary muscle groups
(Figure 7.110), so that all the cords insert

into a common muscle mass46. In the
original description47, in contrast, the
parachute lesion was dened on the basis of
208
Sup.
Right
Left
Inf.
Fig. 7.107 Having shortened the cords of the prolapsed mitral

valve, as shown in Figure 7.106, the surgeon has completed the
repair by inserting an annular ring to support the atrioventricular
junction, which has been reduced by annuloplasty.
Sup.
Right
Left
Inf.
Fig. 7.108 This specimen, seen from the inlet aspect of the left
ventricle in anatomical orientation, has a cleft (arrow) in the
aortic leaet of an otherwise normally structured mitral valve. This
lesion should be distinguished from the space between the left
ventricular bridging leaets, or the so-called cleft, found in the
left atrioventricular valve of hearts with decient
atrioventricular septation and a common atrioventricular junction
(see Figure 7.109).
gross hypoplasia of one end of the zone of

apposition between the leaets, together
with an absence of its supporting papillary
muscle. Irrespective of how the lesion is
dened, surgical reconstruction is
difcult, and valvar replacement is likely to

be necessary48. Parachute deformity of
the mitral valve may be complicated
further by other lesions, such as a
supravalvar left atrial stenosing ring, and
coarctation of the aorta. The combinations

are known as Shones syndrome47.
Parachute malformation of the tricuspid
valve can occur, but is rarely of clinical
signicance49.
209
Sup.
Post.
Ant.
Inf.
Mural leaflet
Fig. 7.109 This heart with decient atrioventricular septation, a

common atrioventricular junction, and shunting exclusively at the
ventricular level, the so-called ostium primum defect, is
photographed from the left side in anatomical orientation. Note
the difference between the zone of apposition between the left
ventricular components of the bridging leaets (arrow), the socalled cleft, and the true cleft of the aortic leaet of an otherwise
normal mitral valve shown in Figure 7.108.
Fig. 7.110 This specimen, removed at surgery, shows the socalled parachute arrangement of the mitral valve. There is fusion
of the papillary muscles, along with thickening and fusion of the
tendinous cords.
210
MALFORMATIONS OF THE
ARTERIAL VALVES AND
OUTFLOW TRACTS
In this section, we consider the surgical
aspects of subvalvar obstruction of the
ventricular outow tracts, valvar stenosis,
and atresia of the outow. In the normally
connected heart, obstruction in the left
ventricular outow tract produces
subaortic stenosis. It must be remembered
that the same anatomical lesions will
produce subpulmonary obstruction in the
patient with discordant ventriculoarterial
connections. Similarly, obstruction of the
right ventricular outow tract produces
subpulmonary obstruction in the heart
with normal segmental connections, but
subaortic stenosis when the
ventriculoarterial connections are
discordant. When both outow tracts are
connected to the same ventricle, the
anatomical problems are more discrete.
These are considered separately in
Chapter 8.
Stenosis of the aortic valve can occur at
valvar, subvalvar, and supravalvar levels.
Aortic regurgitation is ultimately a valvar
problem, and the perivalvar anatomy is
often of great importance. To understand
the substrates for stenosis and regurgitation
across the arterial valves fully, it is essential

to have a rm grasp of the arrangement of
the valvar leaets at the ventriculoarterial
junction. As described in Chapters 2 and 3,
the arterial valves do not possess an annulus
in the sense of a circular ring of collagen
that supports the leaets in the fashion of a
circle. The only ring within the valvar
complex, aside from the sinutubular
junction, is the circular area over which the
brous wall of the great arterial trunk is
supported by the underlying ventricular
structures50. Surgeons do not dene this
ring, however, as the annulus51. The
junctions are partly muscular and partly
brous in the left ventricle
(Figure 7.111)52, but exclusively muscular
at the right ventriculoarterial junction
(Figure 7.112)50. The attachments of the
leaets are arranged as half moons, with the
bases of the leaets attached to ventricular
muscle, but the apex of the leaets attached
to the brous wall of the arterial trunk at
the sinutubular junction. When seen in the
closed position, the three leaets coapt
snugly along their zones of apposition,
which extend from the circumferential
margins of the arterial wall to the centre of
the valve (Figure 7.113). It is on the basis of
perturbation of this coaptation of the
leaets under pressure of the diastolic
column of blood that stenosis or

regurgitation occurs within the valvar
complex.
Valvar aortic stenosis may occur with
unicuspid, bicuspid, tricuspid and, rarely,
even quadricuspid congurations.
Dysplastic lesions are also seen in the aortic
valve, but only rarely can surgery provide
the answer to this problem. The unicuspid
valve has two raphes at the ends of abortive
zones of apposition (Figure 7.114). The
leaets are also abnormally attached in
linear rather than semilunar fashion
(Figure 7.115). Surgical opening of the
raphes in the conjoined leaets typically
results in valvar incompetence. The
creation of new interleaet triangles,
supplementing the leaet tissue so as to
create new semilunar hinges, is likely to
produce greater clinical success.
A valve with two effective leaets is seen
most frequently in the adult patient.
Perhaps this is because such a bicuspid
valve, in itself, is not usually intrinsically
stenotic. It is only with the effects of time
and turbulence that the bifoliate valves
become manifestly obstructive. If the
valvar morphology has not been totally
obscured by calcic deposits, a bicuspid
valve seen at operation will take one of two
forms. Occasionally the two leaets are of
Muscular support
Sup.
Post.
Ant.
Inf.
Fig. 7.111 The aortic outow tract has been spread open and is
Fibrous support
photographed from the ventricular aspect in anatomical

orientation. The arterial valvar leaets are attached in semilunar
fashion, taking their origin in part from brous tissue, and in part
from the muscular ventricular septum.
211
Anatomical ventriculoarterial junction
Fig. 7.112 In this anatomical specimen, the subpulmonary

infundibulum has been opened and photographed in anatomical
orientation, the leaets of the pulmonary valve having been
removed. Note that the semilunar attachments of the leaets cross
the circular anatomical ventriculoarterial junction. This is more
obvious than in the subaortic outow tract (see Fig. 7.111), but the
basic arrangement is comparable.
Muscular support
Fig. 7.113 In this specimen, the aortic valve is photographed from

above in its closed position. The three zones of apposition between
the leaets (red double-headed arrows) extend from the
sinutubular junction at the periphery (stars) to the centre of the
valvar orice (red oval). The leaets are closed by the hydrostatic
pressure of the column of blood they support.
equal size, the solitary zone of apposition

between them bisecting the aortic root
(Figure 7.116). This type of valve is
frequently found in patients with
coarctation of the aorta. In the other form,
the leaets are unequal, with the large, or
conjoined, leaet usually exhibiting a

raphe, which can be eccentrically placed
(Figure 7.117) or centrally positioned
(Figure 7.118) within the conjoined leaet.
If the conjoined leaet is produced by
fusion of the leaets guarding the aortic
sinuses giving rise to the coronary arteries,

then both arteries will arise from the
conjoined sinus. Alternatively, when
the conjoined sinus is formed from the
right coronary and non-coronary aortic
sinuses, the coronary arteries will be
212
Fused zones of apposition
Sup.
Right
Left
Inf.
Fig. 7.114 This view of an abnormal aortic valve seen through an

aortotomy shows the so-called unicuspid and unicommissural
arrangement. Fusion of two of the putative zones of apposition
during development leaves the persisting zone of apposition as the
eccentric valvar orice.
Solitary patent zone of apposition
Fig. 7.115 This picture of the aortic valve, taken in a specimen

Sup.
Post.
Ant.
Mitral valve
Inf.
positioned so that one coronary artery

arises from the conjoined sinuses, and the
other from the third sinus53. It is
increasingly recognised that the two
phenotypes carry signicant differences
with regard to anticipated problems during
with the left ventricular outow tract having been opened, shows
the unicuspid and unicommissural arrangement as demonstrated
in Fig. 7.114. There is loss of the semilunar suspension of the
leaets (black dotted line), so that paradoxically they are
attached in true annular fashion. The solitary zone of apposition
(red arrow) points backwards towards the mitral valve. Note the
marked reduction in height of the remaining interleaet triangles
(white arrows).
follow-up. When the conjoined leaet is

made up of the right and non-coronary
aortic leaets, it is common to nd
degenerative disease of the aortic walls. If
valves with two effective leaets are seen
before they become rigid and distorted by
calcication, some relief from the stenosis

can be obtained by careful enlargement of
the ends of the solitary zone of apposition.
Careful follow-up is essential, especially
when the phenotype points towards
degenerative disease.
213
Sup.
Right
Left
Inf.
Fig. 7.116 This picture, taken in the operating room through

an aortotomy, shows an aortic valve with two leaets of
comparable size.
Sup.
Right
Left
Inf.
Aortic stenosis also occurs in patients

with valves having three leaets, but is not
usually seen until later in life. A possible
cause of such stenosis is the unequal size of
the leaets in the otherwise normal aortic
valve. This, coupled with the high pressure
Fig. 7.117 This picture, taken in the operating room through

an aortotomy, shows a bicuspid aortic valve with a raphe (star) in
one of the leaets.
in the aortic root, may lead to the

development of calcication and stenosis in
the elderly (Figure 7.119)54.
Subvalvar stenosis may be brous,
bromuscular, or muscular, reecting the
fact that the left ventricular outow tract is
partly muscular and partly brous. The

muscular portion comprises the
ventriculoinfundibular fold anterolaterally,
the small outlet component of the muscular
septum anteriorly, and the upper edge of
the apical part of the muscular septum
214
Sup.
Right
Left
Inf.
Fig. 7.118 In this specimen, the aortic valve is photographed

from behind in anatomical orientation, the left ventricular outow
tract through the mitral valve having been opened. The two
leaets arising from the sinuses giving rise to the coronary
arteries have fused, with the line of fusion represented by the
raphe (star).
Sup.
Right
Left
Inf.
Fig. 7.119 This abnormal aortic valve, photographed from the

aortic aspect, has fusion of two of the zones of apposition
(black dotted lines) between the leaets, with calcication also
present. This is the typical substrate of aortic stenosis as seen in the
elderly.
posteriorly. The brous part comprises the

central brous body, the area of continuity
between the leaets of the aortic and mitral
valves, and the left brous trigone.
Subvalvar stenoses may also be either xed
or dynamic in nature.
Of the variants producing xed

stenosis, a subvalvar brous shelf is
perhaps most easily approached
surgically. It appears circular when
viewed through the usually normal aortic
valve (Figure 7.120). Indeed, it can be
circular. This is not always the case. A

relatively thin shelf of tissue sometimes
runs from beneath the non-coronary
leaet of the aortic valve, originating over
the site of the penetrating bundle, then
extending to the septal musculature,
215
Subvalvar fibrous shelf
Sup.
Right
Left
Inf.
Fig. 7.120 This view, taken in the operating room through the
aortic valve, shows a circular brous shelf producing subaortic
stenosis.
Sup.
Right
Left
Inf.
Mitral valve
Fig. 7.121 This specimen is opened anteriorly through the

subaortic outow tract, and is photographed in anatomical
orientation. Note the extensive shelf-like lesion producing
subvalvar stenosis, and extending onto the aortic leaet of the
mitral valve (arrows).
eventually coursing over the

ventriculoinfundibular fold. The shelf can
also extend laterally to involve the aortic
leaet of the mitral valve (Figure 7.121). If
dissection is performed carefully55, taking
particular care where the shelf intimately

overlies the conduction tissues, a
circumferential lesion can be removed
completely (Figure 7.122). Too vigorous
an attack on the side of the mitral valve
may lead to detachment of that structure.

In cases where the ventricular septum
appears to be playing a part in causing the
stenosis, it may be prudent to remove a
segment of muscle (Figure 7.123).
216
Fig. 7.122 This specimen is the subaortic shelf shown in

Figure 7.120 subsequent to its surgical removal. Note the horseshoe
conguration.
Sup.
Right
Left
Inf.
Fig. 7.123 This view, taken in the operating room through the
aorta, shows how a segment of ventricular muscle (arrow) can
be removed safely to relieve shelf-like brous obstruction of the
left ventricular outow tract.
In cases where complete removal proves

difcult, interruption of the brous shelf in
the safe area over the ventriculoinfundibular
fold will result in a safe and satisfactory relief
of the stenosis. The same rules apply when
resecting the variant of aortic stenosis
producing a bromuscular tunnel. Surgical

correction, however, may be less successful
than with a simple shelf, because the tunnel
extends farther into the left ventricle,
making the obstruction it produces more
difcult to relieve.
A much more rare form of xed

subaortic obstruction is produced by
hypertrophy of the usually inconspicuous
anterolateral muscle bundle56. This muscle
runs down the outow tract from the
ventriculoinfundibular fold to the
ventricular septum. In its course over the

parietal wall, it would not be expected to
involve the conduction tissues.
Anomalous attachment of the left
atrioventricular valve can also cause xed
obstruction, as can a deviated muscular
outlet septum. The former is usually seen
with atrioventricular septal defect. The

latter occurs only in the presence of a
ventricular septal defect (Figure 7.124).
The xed type of subaortic obstruction is
also produced by so-called tissue tags. These
can herniate from any adjacent brous tissue
structure, but are exceedingly rare as an
217
isolated lesion in the normally connected

heart57. They can produce signicant
obstruction of the left ventricular outow
tract in hearts with an atrioventricular
septal defect (Figure 7.125), or with
discordant ventriculoarterial connections
(see Chapter 8).
Muscular outlet ventricular septal defect

Deviated muscular outlet septum
Sup.
Fig. 7.124 This anatomical specimen shows the left ventricle

Post.
Ant.
Inf.
Narrowed subaortic outflow tract
opened in clam-shell-like fashion, and viewed from the apex of the

left ventricle. There is a xed subaortic obstruction produced by the
posterior deviation of the muscular outlet septum through a
Sup.
Post.
Ant.
Inf.
Fig. 7.125 This heart is opened through the subaortic outow

tract, and photographed in anatomical orientation. There is an
atrioventricular septal defect with a common atrioventricular
junction, with subaortic obstruction due to tissue tags (arrows).
218
Dynamic subvalvar obstruction is a

result of thickening of the septal
musculature abutting the aortic leaet of
the mitral valve during ventricular systole.
This usually creates a ridge of thickened
endocardium, easily seen through the
aortic valve. If an operation becomes
necessary, the hypertrophied muscle
bundle can be resected, offering
satisfactory relief of the obstruction58.
Again the surgeon must scrupulously avoid
the conduction tissue as it emerges beneath
the zone of apposition between the right
and non-coronary leaets and descends on
the muscular ventricular septum
(Figure 7.126). Interventionists are now
able to offer alternative therapy by injecting
alcohol into the rst septal perforating
artery.
Supravalvar aortic stenosis occurs in
hourglass, membranous, and more diffuse
tubular variants. All forms are rare.
Fortunately, the severe tubular type is
extremely unusual. Two problems are
shared by all three varieties because of
narrowing of the aorta at the junction of the
sinuses with the ascending tubular aorta59.
Firstly, the aortic sinuses, which usually
contain the coronary arteries, may be
converted into high pressure zones, in
which the arteries provide the only run-off
other than through the distal stenosis. This
can produce marked dilation of both the
sinuses and the coronary arteries.

Secondly, the circumferential narrowing at
the sinutubular junction (Figure 7.127)
tends to tether the three aortic leaets at the
ends of their zones of apposition in such a
way that it is rarely enough to perform a
simple aortoplasty60. If possible, all three
sinuses should be opened to release the
tethering of the leaets. This can be
accomplished by resecting the thickened
sinutubular junction, and inserting
pericardial patches in each sinus
(Figures 7.1287.131).
Aortic valvar insufciency may be due
to congenital malformation of the valve
(Figure 7.132), its supporting structures
(Figure 7.133), or both. It may also be
secondary to an infectious process in the
aortic root (Figure 7.134), or to
degenerative disease of the aortic walls.
Occasionally, aortic insufciency may be
due to trauma. Its frequent association
with the doubly committed and juxtaarterial ventricular septal defect suggests
that deciency in the structures
supporting the leaets plays some role in
these problems. Prolapse of the leaets,
and insufciency, may occur with other
types of ventricular septal defect
(Figure 7.135). Prolapse can be found
even when the ventricular septum is
intact, the latter situation usually being
associated with a bicuspid aortic valve.
The critical importance of the anatomy

of this region perhaps is best demonstrated
by the problems exhibited by patients with
endocarditis of the aortic valve61. Because
the valve is the keystone to all the other
valves and chambers of the heart
(Figure 7.136), an eroding abscess in the
aortic root may lead to formation of a stula
involving any of these adjacent structures.
The patient may present with ndings of
left heart failure, left-to-right shunting,
complete heart block, or any combination
of these, in addition to the usual signs of
sepsis. Surgical management requires a
detailed knowledge of this area, as the
surgeon may be faced with virtual
disruption of the ventriculoarterial
connection61. A similar problem can occur
when the aortic root, or the brous coronet,
is severely damaged by dissection or
marked degeneration of its brous
structure.
As with aortic stenosis, stenosis of the
right ventricular outow can occur at the
valvar, supravalvar, or subvalvar levels.
The latter is discussed in association with
tetralogy of Fallot (see later). Dysplasia of
the valvar leaets is most often seen as
marked distortion and thickening, although
three discrete leaets can sometimes be
recognised (Figure 7.137). It can be
associated with insufciency as well as
stenosis.
Mitral
valve
Sup.
Right
Left
Inf.
Left bundle
branch
Tricuspid
valve
Fig. 7.126 The cartoon shows the location of the atrioventricular

conduction axis as it would be visualised by the surgeon working
through the aortic valve.
219
Sup.
Post.
Ant.
orientation, there is severe narrowing at the level of the

sinutubular junction (arrows). Although usually termed
supravalvar, the obstruction involves the attachment of the valvar
leaets (stars) at the sinutubular junction.
Inf.
Aortic root
Pulmonary trunk
Tubular aorta
Left
Inf.
Sup.
Fig. 7.128 This picture, taken in the operating room through a

Right
Isolated pulmonary stenosis typically

is found in the form of a dome-shaped
valve with three well-developed but fused
commissures (Figure 7.138)50. Typically,
median sternotomy, shows narrowing (arrows) of the aorta at the

level of the sinutubular junction.
the leaets are attached to the wall of the

pulmonary trunk along the peripheral
ends of the zones of apposition between
them, leaving only a restricted central
opening (Figure 7.139).The arterial root

is narrowed at the sinutubular junction.
This is an integral part of the valvar
mechanism, although such narrowing is
220
Left coronary aortic leaflet
Left
Fig. 7.129 In the patient shown in Figure 7.128, the surgeon has
Inf.
Sup.
Right
Non-coronary aortic leaflet
made an extensive vertical incision into the non-coronary sinus or

the aortic root, revealing the marked constriction at the
sinutubular junction, with a particularly narrow entry to the left
coronary aortic sinus, which is guarded by the edge of the valvar
leaet.
Left
Inf.
Sup.
Right
often described as being supravalvar.

These areas of tethering can be
dissected from the arterial wall and
incised, providing a particularly
Fig. 7.130 The attachments of the left and non-coronary leaets

at the thickened sinutubular junction, as shown in Figure 7.129,
have been liberated (arrow), thus releasing the left coronary leaet
so as to allow its full excursion.
satisfactory relief of the obstruction. The

effectiveness of surgery is best measured
six to nine months after operation, as
signicant secondary muscular
obstruction at the subvalvar level may

maintain a pressure gradient across the
outow tract. This muscular hypertrophy
will almost always regress with time62.
221
Left
Inf.
Sup.
Fig. 7.131 In the patient shown in Figures 7.129 and 7.130, a

helical pericardial patch (white double-headed arrow) has been
inserted in the incision to the non-coronary sinus, thus enlarging
the sinutubular junction and the ascending aorta.
Right
Sup.
Right
Left
Fig. 7.132 This view, taken in the operating room through an

aortotomy, shows a regurgitant aortic valve as the consequence of
tethering of one of its leaets (arrow).
Inf.
Despite the excellent results of surgery,

treatment of congenital pulmonary valvar
stenosis has become largely the province
of the interventional cardiologist. It is
doubtful, however, whether ination of a

balloon will ever rival the anatomical
precision achieved by the competent
surgeon.
True supravalvar stenosis usually

takes the form of a waist-like narrowing of
the pulmonary trunk distal to the
sinutubular junction (Figure 7.140),
222
Sup.
Right
Left
Inf.
Fig. 7.133 This anatomical specimen is photographed from

above, showing failure of central coaptation of the leaets of the
aortic valve because of dilation at the sinutubular junction.
Sup.
Right
Left
Inf.
Fig. 7.134 This operative view, taken through an aortotomy,

shows a perforation (arrow) in one leaet of a bicuspid aortic valve,
due to infective endocarditis.
223
Pulmonary
trunk
Left
Inf.
Sup.
Fig. 7.135 This operative view, seen through a right

ventriculotomy, shows prolapse of the leaets of the aortic valve
(star) in the setting of a perimembranous ventricular septal defect
opening into the outlet of the right ventricle.
Right
Pulmonary
root
Mitral
valve
Tricuspid
valve
Sup.
Ant.
Post.
Inf.
Fig. 7.136 The short axis of the ventricular mass has been
displayed by removing the atrial musculature and the arterial
trunks, showing the keystone location of the centrally positioned
aortic valve (star).
224
Left
Inf.
Sup.
Fig. 7.137 This operative view, taken through an incision in the

pulmonary trunk, shows gross dysplasia of the leaets of the
pulmonary valve.
Right
Fig. 7.138 This pulmonary valve is viewed from above in

anatomical orientation. There is extensive fusion of the zones of
apposition between the leaets, leaving a domed membrane with
a central orice the size of a pinhole. Note the tethering of the
domed valvar tissue to the walls of the pulmonary trunk at the sites
of fusion of the leaets (arrows).
although it may occur at the

sinutubular junction, or anywhere at
one or more locations within the
pulmonary arterial tree. Narrowing has
also been reported within collateral
arteries supplying the lung directly

from the aorta in cases of tetralogy of
Fallot with pulmonary atresia63. Very
rarely, the obstructions may be
membrane-like, but the usual lesion is
more akin to a segment of

tubular hypoplasia. These lesions, if
anatomically accessible, are
amenable to enlargement using a simple
patch.
225
Left
Inf.
Sup.
Right
Fig. 7.139 This operative view, taken through an incision in the

pulmonary trunk, shows fusion of the zones of apposition between
the leaets of the pulmonary valve, with tethering of the fused
leaets to the wall of the pulmonary trunk (arrows), leaving a
constricted central opening (star).
Pulmonary root
Left
Inf.
Sup.
Right
Fig. 7.140 The view, taken in the operating room, shows the
Pulmonary trunk
external narrowing (arrows) at the sinutubular junction of the

pulmonary valve demonstrated in Figure 7.139.
TETRALOGY OF FALLOT
One form of obstruction of the right
ventricular outow tract is so clearly
demarcated that it constitutes an entity in
its own right, namely tetralogy of Fallot.
When there is extreme overriding of the

aortic valvar orice, it can also show the
abnormal ventriculoarterial connection of a
double-outlet right ventricle, but it is
convenient to discuss the entity at this
point of our narrative. Its anatomical
hallmark is anterocephalad deviation of

the insertion of the muscular or brous
outlet septum, combined with
subpulmonary obstruction due to
hypertrophy of septoparietal
trabeculations64. In the normal heart
226
Sup.
Ant.
Post.
Inf.
Aorta
Tricuspid valve
Septomarginal
trabeculation
(Figure 7.141), the muscular outlet

septum is inconspicuous, being the small
area of the supraventricular crest inserted
between the limbs of the septomarginal
trabeculation. In this position, it is fused
with the much more extensive
ventriculoinfundibular fold, the crest itself
supporting the distal free-standing
infundibular sleeve.
In tetralogy of Fallot, the outlet
part of the septum is malaligned
anterocephalad to the anterior limb of the
septomarginal trabeculation, becoming
recognisable in its own right. The deviated
position of the septum, combined with
hypertrophy of the septoparietal
trabeculations, serves to narrow the
subpulmonary outow tract. At the
same time, it leaves an interventricular
communication that is overridden by
the leaets of the aortic valve
(Figure 7.142).
The interventricular communication,
therefore, is positioned beneath the
ventricular outlets in association with
malalignment of the muscular outlet
septum. Therefore, it is the rightwards
margin of the cone of space subtended by
the overriding valvar leaets that should
be dened as the ventricular septal defect.
There can be brous continuity between
Fig. 7.141 The normal right ventricle is photographed in

anatomical orientation, showing the septal aspect. The
supraventricular crest inserts between the limbs of the
septomarginal trabeculation. Although when, using dissection, it is
possible to create a direct communication with the left
ventricular outow tract through the muscle inserted between the
limbs of the septomarginal trabeculation, it is not possible, in
the normal heart, to distinguish where the septal component
nishes, and where the musculature becomes that of the parietal
ventricular wall.
the leaets of the aortic and tricuspid

valves in the inferocaudal quadrant of the
defect thus dened, making it
perimembranous (Figure 7.143).
Alternatively, the inferocaudal rim
can be muscular when the
ventriculoinfundibular fold fuses with the
caudal limb of the septomarginal
trabeculation (Figure 7.144)65. These
features have the same implications for
protection of the axis of atrioventricular
conduction tissue as they do in isolated
ventricular septal defects (see earlier).
When the inferocaudal margin is brous,
the atrioventricular conduction axis
penetrates beneath the atrioventricular
membranous septal component of this
area (Figure 7.145). Often this is overlain
by the membranous ap, or pseudoaps
derived from the tricuspid valve66.
Usually, in tetralogy of Fallot, the nonbranching and branching components of
the atrioventricular bundle are carried
down the left ventricular side of the
septum, being positioned some distance
from the septal crest. In a minority of
cases, the bundle can branch directly
astride the septum67,68. It can then be
traumatised (Figure 7.146) by sutures
placed directly through the septal crest
(Figure 7.147).
The cases in which the posterior

limb of the septomarginal trabeculation
has fused with the ventriculoinfundibular
fold superior to an intact interventricular
membranous septum (Figure 7.144),
thus protecting the atrioventricular
conduction tissues, account for about
one-fth of the overall population with
this lesion. In this setting, supercial
sutures can be placed along the entire
muscular margin of the right
ventricular aspect of these defects
without fear of traumatising the
conduction axis.
In rare cases, more frequently
encountered in Asia or South America69,70,
the outlet septum is brous rather than
muscular, with failure of formation of the
posterior aspect of the free-standing
subpulmonary infundibular sleeve. The
defect is thus doubly committed and
juxta-arterial (Figure 7.148). The
signicant surgical feature in these cases is
again the relationship of the conduction
axis in the inferior border of the defect,
which may be either muscular or
perimembranous.
While it is clearly important to close
the ventricular septal defect securely in
patients with tetralogy of Fallot,
patching the aorta into the left ventricle,
227
Pulmonary valve
Outlet septum
Ventriculoinfundibular
fold
Aortic valve
Sup.
Ant.
Post.
Inf.
Aortic valve
Fig. 7.142 This view of the outlet of the right ventricle of the
heart from a patient with tetralogy of Fallot, taken in anatomical
orientation, shows the divorce of the muscular structures that
combine to form the normal subpulmonary outow tract.
Pulmonary valve
Fibrous
continuity
Tricuspid valve
Fig. 7.143 This view of an anatomical specimen, photographed in

anatomical orientation, shows the usual variant of the ventricular
septal defect in tetralogy of Fallot, in which the posteroinferior
border is formed by a brous continuity between the leaets of the
aortic and tricuspid valves, thus making it perimembranous. The
star shows the deviated muscular outlet septum, which supports a
sleeve of free-standing subpulmonary infundibular musculature
(arrow).
Sup.
Ant.
Post.
Inf.
probably the most important feature

for a successful surgical outcome, is
relief of the subpulmonary obstruction.
One of the major determinants of this
success is the size of the pulmonary

trunk. Tables are available for
preoperative evaluation to select those
patients who can be corrected
successfully without incising across the

ventriculoarterial junction71,72. Such
incisions are usually described as being
transannular.
228
Outlet septum
fold
Sup.
Ant.
Post.
Inf.
Fig. 7.144 In this specimen with tetralogy of Fallot,

photographed in anatomical orientation, the defect (star), as seen
from the right ventricle, has exclusively muscular borders, because
of fusion posteroinferiorly between the caudal limb of the
septomarginal trabeculation and the ventriculoinfundibular fold.
Membranous flap
Sup.
Ant.
Post.
Inf.
Fig. 7.145 This anatomical specimen with tetralogy of Fallot,

photographed in anatomical orientation, has been prepared by
removal of the septal leaet of the tricuspid valve. It shows the site
of the remnant of the interventricular membranous septum,
known as the membranous ap, and illustrates the relationships of
the atrioventricular conduction axis (red dotted line) when the
ventricular septal defect is perimembranous. The star shows the
229
Patch
Sup.
Fig. 7.146 In this heart, viewed in anatomical orientation from
Post.
Ant.
Inf.
Haematoma on septal crest
the left ventricle, the defect in a patient with tetralogy of Fallot was
repaired by placing sutures directly through the crest of the
muscular ventricular septum. Note the haemorrhage produced at
the septal crest.
Haemorrhage
in AV bundle
Sup.
Fig. 7.147 The histological section is taken across the ventricular
Left.
Right
septum in the heart shown in Figure 7.146. The atrioventricular

(AV) conduction tissue axis in this heart branched directly astride
the septum. The atrioventricular bundle was traumatised by a
suture (arrow) securing the patch, producing atrioventricular block.
Inf.
An understanding of the precise

anatomy of the subpulmonary outow tract
is also vital if the surgeon is to plan a
reproducible operation for successful relief
of the muscular obstruction accurately.
This is the consequence of formation of a
constrictive muscular ring at the mouth of
the subpulmonary infundibulum, with its

parietal segment produced by hypertrophy
of free-standing septoparietal
trabeculations. Knowledge of this feature is
important to the surgeon when deciding
which muscle to resect so as to widen the
narrowed outow tract. The major limiting
structure is always the hypertrophied

septal insertion of the outlet septum, which
can be incised without fear of damaging
vital structures. At the same time, any
free-standing septoparietal trabeculations
should be identied and removed
(Figures 7.149, 7.150). They, too, never
230
Pulmonary
trunk
Aorta
Fig. 7.148 This specimen, viewed in anatomical orientation from
Sup.
Left.
Right
Inf.
Aortic-pulmonary
continuity
Perimembranous VSD
Outlet septum
Left
the apex of the right ventricle, shows tetralogy of Fallot with a

doubly committed and juxta-arterial defect due to failure of
formation of the muscular subpulmonary infundibulum. Note the
valves, showing that the defect is also perimembranous. VSD,
Septoparietal
trabeculation
Apex
Base
Right
Fig. 7.149 This view, taken in the operating
Infundibular orifice
Pulmonary valve
contain vital structures. The body of the

outlet septum usually contributes to
obstruction, and it is tempting to resect this
structure. Excessive resection, however,
may lead to damage to the leaets of the
aortic valve arising from its left ventricular

aspect.
It is also usual to resect the parietal
insertion of the outlet septum (Fig. 7.151).
This fuses with the ventriculoinfundibular
room through a right infundibulotomy, shows

the stenotic orice of the subpulmonary
infundibulum in a patient with tetralogy of
Fallot. The stenosis is formed in part by the
hypertrophied outlet septum, and also by
septoparietal trabeculations.
fold, which is the inner curvature of the

heart. Care must be taken not to perforate
to the right-sided atrioventricular groove in
this region. Dissection, or injudicious
placement of sutures, can damage the
231
Septoparietal trabeculations
Left
Apex
Base
Right
Fig. 7.150 In the patient shown in

Figure 7.149, the surgeon was able to liberate
the septoparietal trabeculations. They could
then be excised.
Infundibular orifice
Outlet septum
Resected
septoparietal
bands
Left
Apex
Base
Resected parietal attachment
of septal insertion
Right
right coronary artery73. It is very unusual

for the septomarginal trabeculation itself
to contribute to the subpulmonary
obstruction. Thus, it is usually unnecessary
to resect its limbs. Its body, and the
moderator band, nonetheless, may be
Fig. 7.151 In the patients shown in Figures 7.149 and 7.150, the
obstruction at the mouth of the infundibulum was
completely relieved by resecting the parietal extension of the
outlet septum.
hypertrophied, particularly when the latter

structure has a high take-off. Severe
hypertrophy produces a two-chambered
right ventricle74, when the intervening
muscle band may require resection
(Figure 7.152). The anterior papillary
muscle of the tricuspid valve often arises

from the inlet aspect of the obstructing
shelf. Care must be taken, therefore, not to
damage this muscle during resection.
The nal variable in tetralogy of Fallot is
the connection of the leaets of the
232
Sup.
Fig. 7.152 In this heart from a patient with tetralogy of Fallot,
Ant.
Post.
shown in anatomical orientation, there is hypertrophy of the body

of the septomarginal trabeculation (star), separating the apical
trabecular component into two parts (arrows). This is the so-called
two-chambered right ventricle.
Inf.
overriding aortic valve. Depending on this

feature, the aorta can be connected mostly to
the left ventricle, making the
ventriculoarterial connection concordant, or
connected mostly to the right ventricle, and
hence producing a double-outlet
connection. The degree of override should
not affect the surgical procedure markedly.
With greater commitment of the aorta to the
right ventricle, nonetheless, the placement
of the patch tunnelling the aorta to the left
ventricle, and closing the ventricular septal
defect, becomes more important. The
internal conduit constructed from the left
ventricle to the aorta may complicate relief
of the obstruction to the right ventricular
outow tract further, because it is always
necessary to ensure an adequate outlet from
the left ventricle.
Although the primary obstruction in
tetralogy of Fallot is at the infundibular
level, the pulmonary valve is frequently
stenotic, being bifoliate in the majority
of patients. Any stenosis must be
relieved during operative repair. The
sequels of postoperative pulmonary
regurgitation only now are becoming
evident. It could be that, in the long term,
they will be just as troublesome as residual
pulmonary stenosis, and less easy to relieve.
Patients with tetralogy of Fallot can

have multiple associated lesions, such as
valve, or decient atrioventricular
septation. The end point is then a
combination of the anatomical lesions
already discussed. One of the most
important associated lesions is the
presence of pulmonary atresia rather than
pulmonary stenosis. This combination is
often described as pulmonary atresia
with ventricular septal defect, which is
not an incorrect designation. The variant
found with the intracardiac anatomy of
the tetralogy, however, is so distinctive
that it should be described as tetralogy of
Fallot with valvar or infundibular
pulmonary atresia75. The intracardiac
anatomy includes deviation of the
muscular outlet septum sufcient to
block the subpulmonary infundibulum
completely (Figure 7.153). The anatomy
of the ventricular septal defect can vary as
in tetralogy of Fallot, with the same
surgical connotations. The feature that
dominates the surgical options is the
morphology of the pulmonary arteries.
Exceedingly rarely, the pulmonary
arteries may be supplied through a
persistent fth arch76, an aortopulmonary
window, or coronary arterial stulas77. In

most instances, nonetheless, the lungs are
supplied either through a persistently
patent arterial duct (Figure 7.154), or
through major aortopulmonary collateral
arteries78. It is rare to nd such collateral
arteries supplying the pulmonary
circulation except when the intracardiac
anatomy is that of tetralogy of Fallot.
Additionally, only very rarely, will a
duct and collateral arteries supply the
same lung, although these two sources of
ow can supply separate lungs
(Figure 7.155). When a duct is present,
and the pulmonary arteries are conuent,
the arteries supply the entirety of the
lung parenchyma (Figure 7.155),
although they may be variably
developed. The degree of hypoplasia
determines whether total correction is
feasible.
When the right and left pulmonary
arteries are not conuent, or when major
aortopulmonary collateral arteries are
present, the situation is more complex.
Non-conuent pulmonary arteries can be
supplied independently by bilateral ducts,
or one lung can be supplied by a duct and
the other through collateral arteries
(Figure 7.156). It is more usual for
233
Pulmonary
atresia
Sup.
Ant.
Post.
Inf.
Aorta
Fig. 7.153 In this heart from a patient with tetralogy of Fallot

Blind-ending
subpulmonary
infundibulum
with pulmonary atresia, viewed in anatomical orientation, there

are conuent pulmonary arteries supplied by an arterial duct. Note
the extreme anterocephalad deviation of the muscular outlet
septum (star), with muscular atresia at the ventriculoarterial
junction.
Arterial duct
Aorta
Sup.
Left
Right
Inf.
Confluent pulmonary arteries
collateral arteries to supply both lungs with

no duct being present.
Well-developed conuent pulmonary
arteries usually coexist within the
pericardial sac even when there are
Fig. 7.154 The computed tomographic angiogram from a

patient with tetralogy of Fallot and pulmonary atresia shows
conuent pulmonary arteries fed through a persistently patent
arterial duct.
collateral arteries (Figure 7.157). It is

important to establish how much of the
pulmonary parenchyma is connected to the
intrapericardial pulmonary arteries, and
how much is supplied directly by collateral
arteries. It is known that collateral arteries

can anastomose with the pulmonary
arteries at the hilum (Figure 7.158), or
extend into the parenchyma to supply lobar
or segmental arteries directly.
234
Sup.
Fig. 7.155 In this specimen of tetralogy of Fallot with pulmonary

atresia, viewed from behind, the pulmonary arteries are
discontinuous. The left pulmonary artery was initially supplied by
an arterial duct, which has become ligametous (star). The right lung
is supplied through systemic-to-pulmonary collateral arteries
(arrows).
Right
Left
Inf.
Sup.
Left
Right
Inf.
Aorta
Fig. 7.156 The pulmonary arteries in the heart shown in

Figure 7.153, fed by the arterial duct (star), have a normal
segmental distribution.
Intersegmental anastomoses also occur.

The object of preoperative evaluation,
therefore, should be to establish precisely
how much of each lung is supplied by the
intrapericardial pulmonary arteries, as
this is the ultimate determinant of the

success of any attempted total correction.
Cases can be found with supply
exclusively from collateral arteries
(Figure 7.159). In this setting, the arterial
trunk is best described as a solitary

structure (see later).
In contrast to tetralogy of Fallot with
pulmonary atresia, where initial survival is
good, and the results of surgery are
235
Sup.
Aorta
Left
Right
Inf.
Collateral arteries
Fig. 7.157 The computed tomographic angiogram from a patient

Atretic pulmonary trunk
Confluent pulmonary arteries
with tetralogy of Fallot and pulmonary atresia shows the presence

of both systemic-to-pulmonary collateral arteries and pulmonary
arteries.
Sup.
Left
Right
Inf.
continually improving, attempted surgical

correction of patients with pulmonary atresia
and an intact ventricular septum continues to
be disappointing. The anatomy of the lesion
itself accounts for the dismal outcome7981.
Fig. 7.158 In this heart from a patient with tetralogy of Fallot and
pulmonary atresia, shown in anatomical orientation, the
intrapericardial arteries extending to enter the right lung (arrow)
are supplied by a large systemic-to-pulmonary collateral
artery (star).
The atresia can be due either to an

imperforate pulmonary valvar membrane
(Figure 7.160), or to muscular infundibular
atresia. In the latter situation, the pulmonary
trunk is blind-ending, with no vestiges of
leaets of the pulmonary valve

(Figure 7.161). The cavities of the outlet and
trabecular component parts of the right
ventricle are more-or-less completely
obliterated by gross hypertrophy of the
236
Sup.
Aorta
Left
Right
Inf.
Collateral arteries
Fig. 7.159 The computed tomographic angiogram from a patient

with tetralogy of Fallot and pulmonary atresia shows the systemicto-pulmonary collateral arteries arising from the descending aorta
that supply the entirety of the pulmonary parenchyma. In this
patient, there was absence of the intrapericardial pulmonary
arteries.
Ant.
Right
Left
Post.
ventricular wall. In consequence, the cavity is

effectively represented by only the
hypoplastic inlet portion (Figure 7.162).
This cavity is unlikely ever to perform a
Fig. 7.160 In this heart from a patient with pulmonary atresia and
an intact ventricular septum, viewed from above, dissection of the
ventriculopulmonary junction shows an imperforate pulmonary
valvar membrane.
useful function, particularly when stulous

communications extend to the coronary
arteries. The right ventricle probably should
be disregarded when deciding surgical
treatment. When there is a pulmonary valve

present, but its leaets are imperforate, a
spectrum is seen in terms of the size of the
right ventricular cavity82. In some hearts,
237
Ant.
Right
Left
Fig. 7.161 In this heart from a patient with pulmonary atresia

and an intact ventricular septum, there is no evidence of valvar
tissue in the blind-ending pulmonary root (compare with
Figure 7.160).
Post.
Sup.
Left
Right
Inf.
Inlet
Fig. 7.162 In this heart from a patient with pulmonary atresia and
an intact ventricular septum, viewed anatomically, the cavity of the
right ventricle is effectively represented by the inlet component
alone, due to gross hypertrophy of the walls of the apical trabecular
and outlet components. Note the stulous communication
extending through the wall to the anterior interventricular
coronary artery (white arrow).
hypertrophy of the right ventricular

myocardium obliterates the apical trabecular
part of the cavity (Figure 7.163). It is
questionable if these ventricles will ever grow
and become useful, although attempts have
been made to resect apical trabeculations and
rehabilitate the ventricle83. In the most

favourable situation, the cavity is less
hypoplastic, and has well-developed
inlet, apical, and outlet components
(Figure 7.164). These cases are those
which are most amenable to total operative
correction, although increasingly they

are treated by the interventional cardiologist,
who will perforate the valvar membrane
before dilating it with balloons.
Whatever the intracardiac anatomy, it is
rare that one nds the thread-like
238
Outlet
Obliterated apex
Inlet
Sup.
Left
Right
Inf.
Fig. 7.163 In this heart, again from a patient with pulmonary

atresia and an intact ventricular septum, mural hypertrophy has
effectively obliterated the apical trabecular component of the
cavity, while leaving narrowed inlet and outlet components.
Mild mural hypertrophy
Outlet
Inlet
Sup.
Left
Right
Inf.
pulmonary arteries seen so frequently with

tetralogy of Fallot and pulmonary atresia.
Furthermore, the ow of pulmonary blood
is almost always duct dependent. With
prostaglandins now available to improve
ductal ow, the pulmonary arteries are
almost always of sufcient size to permit
Fig. 7.164 This heart is once more from a patient having

pulmonary atresia with an intact ventricular septum. It shows the
most favourable variant, in which the pulmonary valve is
imperforate (arrow), but the mural hypertrophy has barely
obliterated any of the right ventricular cavity.
construction of a systemic-pulmonary
shunt. Other options, such as the need for
pulmonary valvotomy, should be decided
after assessment of the precise anatomy of
the individual case.
Pulmonary valvar insufciency may be
congenital or acquired, the latter usually
secondary to surgical intervention or

pulmonary hypertension. Congenital
pulmonary valvar insufciency may be
associated with marked deformity of the
valvar tissue, as in valvar dysplasia, or with
purported absence of the valvar tissue
altogether. In reality, rudimentary leaets
239
Sup.
Left
Right
Fig. 7.165 In this specimen, seen in anatomical orientation,

from a patient with tetralogy of Fallot, there is rudimentary
formation of the leaets of the pulmonary valve (arrow), with gross
dilation of the pulmonary trunk and pulmonary arteries (stars).
Inf.
Left
Apex
Base
Right
Fig. 7.166 This operative view shows the right

ventriculopulmonary arterial junction in a patient with tetralogy of
Fallot. Note the grossly abnormal leaets of the pulmonary valve
(arrows).
are almost always present in the so-called

absent pulmonary valve syndrome. This
can rarely be seen with an intact ventricular
septum84,85, but more usually in
combination with a ventricular septal

defect (Figure 7.165). It is another of the
associated lesions found in combination
with tetralogy of Fallot.
While gross pulmonary valvar

insufciency may be relatively well
tolerated by the right heart, it can
result in marked enlargement of the
240
Left
Apex
Base
Right
Pulmonary trunk
Aorta
Fig. 7.167 In the patient shown in Figure 7.166, there was gross
dilation of the pulmonary trunk and the pulmonary arteries.
Sup.
Left
Right
Inf.
Fig. 7.168 This posterior view of the hilum of the left lung of the
heart from the patient seen in Fig. 7.165 shows the obstruction
produced by the gross dilation of the left pulmonary artery (star) as
it crosses the narrowed bronchus (double-headed arrow).
pulmonary trunk and arteries, and

usually is associated with an absence
of the arterial duct85. Compromise of
the tracheobronchial tree by these
grossly enlarged vessels
(Figures 7.1667.169) results in

most patients presenting with
symptoms of respiratory distress.
Because only a limited number of cases
have come to surgical correction, the
efcacy of replacement of the valve with

or without arterial plication has not
been proved. Perhaps fortunately, socalled absence of the pulmonary valvar
leaets is a rare condition.
241
Pulmonary trunk
Aorta
Left
Base
Apex
Fig. 7.169 This operative view of the patient shown in

Right
References cited
1. Anderson RH, Brown NA. The anatomy
of the heart revisited. Anat Rec 1996;
246: 17.
2. Anderson RH, Webb S, Brown NA. Clinical
anatomy of the atrial septum with reference
to its developmental components. Clin Anat
1999; 12: 362374.
3. Sharratt GP, Webb S, Anderson RH. The
vestibular defect: an interatrial
communication due to a deciency in the
atrial septal component derived from the
vestibular spine. Cardiol Young 2003; 13:
184190.
4. Anderson RH, Ho SY, Falcao S, Daliento L,
Rigby ML. The diagnostic features of
atrioventricular septal defect with common
atrioventricular junction. Cardiol Young
1998; 8: 3349.
5. Crystal MA, Al Najashi K, Williams WG,
Redington AN, Anderson RH. Inferior sinus
venosus defect: echocardiographic diagnosis
and surgical approach. J Thorac Cardiovasc
Surg 2009; 137: 13491355.
6. Butts RJ, Crean AM, Hlavacek AM, et al.
Veno-venous bridges: the forerunners of the
sinus venosus defect. Cardiol Young 2011;
21: 623630.
7. Ettedgui JA, Siewers RD, Anderson RH,
Zuberbuhler JR. Diagnostic
echocardiographic features of the sinus
venous defect. Br Heart J 1990;
64: 329331.
Figures 7.166 and 7.167 shows the grossly enlarged left pulmonary
artery, which is as large as the aorta.
8. Al Zaghal AM, Li J, Anderson RH, et al.

Anatomic criteria for the diagnosis of sinus
venosus defects. Heart 1997; 78: 298304.
9. Knauth A, McCarthy KP, Webb S, et al.
Interatrial communication through the
mouth of the coronary sinus defect. Cardiol
Young 2002; 12: 364372.
10. Warden HE, Gustafson RA, Tarnay TJ,
Neal WA. An alternative method for repair
of partial anomalous venous connection to
the superior vena cava. Ann Thorac Surg
1984; 38: 601605.
11. Stewart RD, Bailliard F, Kelle AM, et al.
Evolving surgical strategy for sinus venosus
atrial septal defect: effect in sinus node
function and late venous obstruction. Ann
Thorac Surg 2007; 84: 16511658.
12. Becker AE, Anderson RH. Atrioventricular
septal defects. Whats in a name?
461469.
13. Gerbode F, Hultgren H, Melrose D,
Osborn J. Syndrome of left
ventricularright atrial shunt, successful
surgical repair of defect in ve cases with
observation of bradycardia on closure. Ann
Surg 1958; 148: 433446.
14. Kelle AM, Young L, Kaushal S, et al. The
Gerbode defect: the signicance of a left
ventricular to right atrial shunt. Cardiol
Young 2009; 19(Suppl 2): 9699.
15. Lacour-Gayet F, Campbell DN, Mitchell
M, Malhotta S, Anderson RH. Surgical
repair of atrioventricular septal defect with
16.
17.
18.
19.
20.
21.
22.
common atrioventricular junction. Cardiol

Young 2006; 16(Suppl 3): 5258.
Wilcox BR, Anderson RH, Henry GW,
Mattos SS. Unusual opening of coronary
sinus in atrioventricular septal defects. Ann
Thorac Surg 1990; 50: 767770.
Penkoske PA, Neches WH, Anderson RH,
Zuberbuhler JR. Further observations on
the morphology of atrioventricular septal
defects. J Thorac Cardiovasc Surg 1985; 90:
611622.
Rastelli GC, Kirklin JW, Titus JL.
Anatomic observations on complete form of
persistent common atrioventricular canal
with special reference to atrioventricular
valves. Proc Staff Meet Mayo Clin 1966; 41:
296308.
Sigfusson G, Ettedgui JA, Silverman NH,
Anderson RH. Is a cleft in the anterior
leaet of an otherwise normal mitral valve
an atrioventricular canal malformation?
J Am Coll Cardiol 1995; 26: 508515.
Thiene G, Wenink ACG, Frescura C, et al.
The surgical anatomy of conduction tissues
in atrioventricular defects. J Thorac
Cardiovasc Surg 1981; 82: 928937.
Wilcox BR, Jones DR, Frantz EG, et al. An
anatomically sound, simplied approach to
repair of complete atrioventricular septal
defect. Ann Thorac Surg 1997; 64: 487494.
Nicholson IA, Nunn GR, Sholler GF, et al.
Simplied single patch technique for the
repair of atrioventricular septal defect. J
Thorac Cardiovasc Surg 1999; 118: 642646.
242
23. Karl TR, Provenzano SC, Nunn GR,

Anderson RH. The current surgical
perspective to repair of atrioventricular
septal defect with common atrioventricular
junction. Cardiol Young 2010; 20(Suppl 3):
120127.
24. Ebels T, Anderson RH, Devine WA, et al.
Anomalies of the left atrioventricular valve
and related ventricular septal morphology
in atrioventricular septal defects. J Thorac
25. Piccoli GP, Ho SY, Wilkinson JL, et al.
Left sided obstructive lesions in
atrioventricular septal defects. J Thorac
26. Pillai R, Ho SY, Anderson RH,
Shinebourne EA, Lincoln C. Malalignment
of the interventricular septum with
atrioventricular septal defect: its
implications concerning conduction tissue
disposition. Thorac Cardiovasc Surg 1984;
32: 13.
27. Milo S, Ho SY, Macartney FJ, et al.
Straddling and overriding atrioventricular
valves morphology and classication. Am
J Cardiol 1979; 44: 11221134.
28. Soto B, Becker AE, Moulaert AJ, Lie JT,
Anderson RH. Classication of ventricular
septal defects. Br Heart J 1980; 43:
332343.
29. Wells WJ, Lindesmith GG. Ventricular
septal defect. In: Arciniegas E (ed).
Pediatric Cardiac Surgery. Chicago, IL:
Year Book Medical Publishers, 1985.
30. Van Praagh R, Geva T, Kreutzer J.
Ventricular septal defects: how shall we
describe, name and classify them? J Am Coll
Cardiol 1989; 14: 12981299.
31. Milo S, Ho SY, Wilkinson JL, Anderson
RH. The surgical anatomy and
atrioventricular conduction tissues of hearts
with isolated ventricular septal defects.
JThorac Cardiovasc Surg 1980; 79: 244255.
32. Van Praagh R, McNamara JJ. Anatomic
types of ventricular septal defect with aortic
insufciency. Diagnostic and surgical
considerations. Am Heart J 1968; 75:
604619.
33. Tsang VT, Hsai TY, Yates R, Anderson
RH. Surgical repair of multiple defects
within the apical part of the muscular
ventricular septum. Ann Thorac Surg 2002;
73: 5862.
34. Kawashima Y, Danno M, Shimizu Y,
Matsuda H, Miyamoto T. Ventricular
septal defect associated with aortic
insufciency: anatomic classication and
method of operation. Circulation 1973; 47:
10571064.
35. Spicer DE, Anderson RH, Backer CL.
Clarifying the surgical morphology of inlet
36.
37.
38.
39.
40.
41.
42.
43.
44.
45.
46.
47.
ventricular septal defects. Ann Thor Surg

2013; 95: 236241.
Pacico AD, Soto B, Bargeron LMJ.
Surgical treatment of straddling tricuspid
valves. Circulation 1979; 60: 655664.
Chauvaud SM, Mihaileanu SA, Gaer JAR,
Carpentier AC. Surgical treatment of
Ebsteins malformationthe Hpital
Broussais experience. Cardiol Young 1996;
6: 411.
Schreiber C, Cook A, Ho SY, Augustin N,
Anderson RH. Morphologic spectrum of
Ebsteins malformation: revisitation relative
to surgical repair. J Thorac Cardiovasc Surg
1999: 117: 148155.
Leung MP, Baker EJ, Anderson RH,
Zuberbuhler JR. Cineangiographic
spectrum of Ebsteins malformation: its
relevance to clinical presentation and
outcome. J Am Coll Cardiol 1988; 11:
154161.
Ruschhaupt DG, Bharati S, Lev M. Mitral
valve malformation of Ebstein type in
absence of corrected transposition. Am
J Cardiol 1976; 38: 109112.
Leung M, Rigby ML, Anderson RH, Wyse
RKH, Macartney FJ. Reversed off-setting
of the septal attachments of the
atrioventricular valves and Ebsteins
malformation of the morphologically mitral
valve. Br Heart J 1987; 57: 184187.
Becker AE, Becker MJ, Edwards JE.
Pathologic spectrum of dysplasia of the
tricuspid valve, features in common with
Ebsteins malformation. Arch Pathol 1971;
91: 167178.
Oberhoffer R, Cook AC, Lang D, et al.
Correlation between echocardiographic and
morphological investigations of lesions of
the tricuspid valve diagnosed during fetal
life. Br Heart J 1992; 68: 580585.
Van der BelKahn J, Duren DR, Becker
AE. Isolated mitral valve prolapse: chordal
architecture as an anatomic basis in older
patients. J Am Coll Cardiol 1985; 5:
13351340.
Layman TE, Edwards JE. Anomalous
mitral arcade: a type of congenital mitral
insufciency. Circulation 1967; 35:
389395.
Rosenquist GC. Congenital mitral valve
disease associated with coarctation of the
aorta. A spectrum that includes parachute
deformity of the mitral valve. Circulation
1974; 49: 985993.
Shone JD, Sellers RD, Anderson RC, et al.
The developmental complex of parachute
mitral valve, supravalvular ring of left
atrium, subaortic stenosis, and coarctation
of the aorta. Am J Cardiol 1963; 11:
714725.
48. Chauvaud S, Mihaileanu S, Gaer JAR,

Carpentier A. Surgical treatment of
congenital mitral valve stenosis. The
Hpital Broussais experience. Cardiol
Young 1997; 7: 1521.
49. Milo S, Stark J, Macartney FJ, Anderson
RH. Parachute deformity of the tricuspid
valve (case report). Thorax 1979; 34:
543546.
50. Stamm C, Anderson RH, Ho SY. Clinical
anatomy of the normal pulmonary root
compared with that in isolated
pulmonary valvular stenosis. J Am Coll
Cardiol 1998; 31: 14201425.
51. Sievers HH, Hemmer G, Beyersdorf F,
et al. The everyday used nomenclature of
the aortic root components : The Tower of
Babel? Eur J Cardiothoracic Surg 2012;
41:478482
52. Anderson RH. Clinical anatomy of the
aortic root. Heart 2000; 84: 670673.
53. Angelini A, Ho SY, Anderson RH, et al.
The morphology of the normal aortic valve
as compared with the aortic valve having
two leaets. J Thorac Cardiovasc Surg 1989;
98: 362367.
54. Vollebergh FEMG, Becker AE. Minor
congenital variations in cusp size in
tricuspid aortic valves. Possible link with
isolated aortic stenosis. Br Heart J 1977;
39: 10061011.
55. McKay R, Ross DN. Technique for the
relief of discrete subaortic stenosis.
917920.
56. Moulaert AJ, OppenheimerDekker A.
Anterolateral muscle bundle of the left
ventricle, bulboventricular ange and
subaortic stenosis. Am J Cardiol 1976; 37:
7881.
57. Anderson RH, Lenox CC, Zuberbuhler JR.
Morphology of ventricular septal defect
associated with coarctation of the aorta.
Br Heart J 1983; 50: 176181.
58. Morrow AG. Hypertrophic subaortic
stenosis: operative methods utilised to
relieve left ventricular outow obstruction.
423430.
59. Stamm C, Li J, Ho SY, Redington AN,
Anderson RH. The aortic root in
supravalvar aortic stenosis: the
potential surgical relevance of morphologic
ndings. J Thorac Cardiovasc Surg 1997;
114: 1624.
60. Doty DB, Polansky DB, Jenson CB.
Supravalvular aortic stenosis. Repair by
extended aortoplasty. J Thorac Cardiovasc
Surg 1977; 74: 362371.
61. Frantz PJ, Murray GF, Wilcox BR.
Surgical management of left
62.
63.
64.
65.
66.
67.
68.
69.
70.
ventricularaortic discontinuity
complicating bacterial endocarditis. Ann
Thorac Surg 1980; 29: 17.
Gilbert JW, Morrow AG, Talbert JW. The
surgical signicance of hypertrophic
infundibular obstruction accompanying
valvar pulmonary stenosis. J Thorac
Haworth SG, Macartney FJ. Growth and
development of pulmonary circulation in
pulmonary atresia with ventricular septal
defect and major aortopulmonary collateral
arteries. Br Heart J 1980; 44: 1424.
Anderson RH, Tynan M. Tetralogy of
Fallota centennial review. Int J Cardiol
1988; 21: 219232.
Anderson RH, Allwork SP, Ho SY, Lenox
CC, Zuberbuhler JR. Surgical anatomy of
tetralogy of Fallot. J Thorac Cardiovasc
Surg 1981; 81: 887896.
Suzuki A, Ho SY, Anderson RH, Deaneld
JE. Further morphologic studies on
tetralogy of Fallot, with particular emphasis
on the prevalence and structure of the
membranous ap. J Thorac Cardiovasc Surg
1990; 99: 528535.
Titus JL, Daugherty GW, Edwards JE.
Anatomy of the atrioventricular conduction
system in ventricular septal defect.
Circulation 1963; 28: 7281.
Anderson RH, Monro JL, Ho SY, Smith A,
Deverall PB. Les voies de conduction
auriculoventriculaires dans le tetralogie de
Fallot. Coeur 1977; 8: 793807.
Ando M. Subpulmonary ventricular septal
defect with pulmonary stenosis. Letter to
Editor. Circulation 1974; 50: 412.
Neirotti R, Galindez E, Kreutzer G, et al.
Tetralogy of Fallot with subpulmonary
71.
72.
73.
74.
75.
76.
77.
ventricular septal defect. Ann Thorac Surg

1978; 25: 5156.
Blackstone EH, Kirklin JW, Bertranou EG,
et al. Preoperative prediction from
cineangiograms of postrepair right
ventricular pressure in tetralogy of Fallot.
542552.
Kirklin JW, Blackstone EH, Pacico AD,
Brown RN, Bargeron LMJr. Routine
primary repair versus twostage repair of
tetralogy of Fallot. Circulation 1979; 60:
373385.
McFadden PM, Culpepper WS, Ochsner J.
Iatrogenic right ventricular failure in
tetralogy of Fallot repairs: reappraisal of a
distressing problem. Ann Thorac Surg 1982;
33: 400402.
Alva C, Ho SY, Lincoln CR, Rigby ML,
Wright A, Anderson RH. The nature of the
obstructive muscular bundles in double
chambered right ventricle. J Thorac
Cardiovasc Surg 1999; 117: 11801189.
Aleri OA, Blackstone EH, Kirklin JW,
et al. Surgical treatment of tetralogy of
Fallot with pulmonary atresia. J Thorac
Macartney FJ, Scott O, Deverall PB.
Haemodynamic and anatomical
characteristics of pulmonary blood supply
in pulmonary atresia with ventricular
septal defectincluding a case of persistent
fth aortic arch. Br Heart J 1974; 36:
10491060.
Pahl E, Fong L, Anderson RH, Park SC,
Zuberbuhler JR. Fistulous communications
between a solitary coronary artery and the
pulmonary arteries as the primary source of
pulmonary blood supply in tetralogy of
78.
79.
80.
81.
82.
83.
84.
85.
243
Fallot with pulmonary valve atresia. Am

J Cardiol 1989; 63: 140143.
Rossi RN, Hislop A, Anderson RH,
Maymone Martins F, Cook AC.
Systemictopulmonary blood supply in
tetralogy of Fallot with pulmonary atresia.
Cardiol Young 2002; 12: 373388.
Freedom RM, Dische MR, Rowe RD. The
tricuspid valve in pulmonary atresia with
intact ventricular septum. A morphological
study of 60 cases. Arch Pathol Lab Med
1978; 102: 2831.
Zuberbuhler JR, Anderson RH.
Morphological variations in pulmonary
atresia with intact ventricular septum.
Br Heart J 1979; 41: 281288.
Anderson RH, Anderson C, Zuberbuhler
JR. Further morphologic studies on hearts
with pulmonary atresia and intact ventricular
septum. Cardiol Young 1991; 1: 105114.
Bull C, de Leval MR, Mercanti C,
Macartney FJ, Anderson RH. Pulmonary
atresia with intact ventricular septum: a
revised classication. Circulation 1982; 66:
266271.
Pawade A, Capuani A, Penny DJ, Karl TR,
Mee RB. Pulmonary atresia with intact
ventricular septum: surgical management
based on right ventricular infundibulum.
J Card Surg 1993; 8: 371383.
Macartney FJ, Miller GAH. Congenital
absence of the pulmonary valve. Br Heart J
1970; 32: 483490.
Emmanouilides GC, Thanopoulos B, Siassi
B, Fishbein M. Agenesis of ductus
arteriosus associated with the syndrome
of tetralogy of Fallot and absent
pulmonary valve. Am J Cardiol 1976; 37:
403409.
Lesions in hearts
with abnormal
segmental
connections
Abnormal segmental connections
In the previous chapter, we paid attention

to those hearts in which the associated
cardiac malformation existed in the setting
of normal connections between the cardiac
segments. All those associated lesions, of
course, can also be found in hearts with
abnormal segmental connections. It is these
abnormal connections that will be our focus
in this chapter, emphasising the associated
anomalies that are particularly frequent
with a given abnormal segmental
arrangement. We conclude the chapter
with a brief discussion of those hearts in
which the relationships of the arterial
trunks are abnormal in the setting of
concordant ventriculoarterial connections,
as these combinations still produce
problems in understanding and
description.
DOUBLE-INLET VENTRICLE
Over the years, hearts with a double-inlet
ventricle have represented one of the
greater challenges to surgical correction.
They have also posed signicant problems
in adequate description and categorisation.
Even these days, many continue to describe
the lesions in terms of single ventricles, or

univentricular hearts, despite the fact that
almost all patients with a double-inlet
atrioventricular connection have two
chambers within their ventricular mass,
one being large and the other small13. The
semantic problems with description can
now be resolved by the simple expedient of
describing functionally univentricular
hearts4, this approach also accounting for
the other lesions dominated by ventricular
imbalance. At this point, we describe the
relevant anatomical characteristics of all
those hearts unied by the presence of the
double-inlet atrioventricular connection,
irrespective of whether they contain one or
two ventricles, although almost all do have
one big and one small ventricle. The
previous problems with description
centred on whether the small chamber in
hearts with ventricular imbalance deserved
ventricular status. We now describe all
chambers possessing characteristic apical
components as ventricles, recognising
them as being complete or incomplete
depending on their component make-up.
This form of analysis greatly simplies the
description of the communications
245
between dominant and incomplete

ventricles, which are no more and no less
than ventricular septal defects.
In basic terms, hearts exhibit a doubleinlet atrioventricular connection whenever
the greater parts of both atrioventricular
junctions, which belong to the right-sided
and left-sided atrial chambers, are
connected to the same ventricle. This
denition holds good irrespective of
whether the connections are guarded by
two separate atrioventricular valves, as is
usually the case (Figure 8.1), or by a
common valve (Figure 8.2). The doubleinlet connection can also be found in the
presence of overriding and straddling
atrioventricular valves, but only when the
degree of overriding is such as to leave the
greater part of both junctions connected to
the same ventricle, again either through
two valves (Figure 8.3) or a common valve.
When there is a straddling common valve,
it becomes moot as to whether the hearts
should also be considered as possessing
atrioventricular septal defects with gross
ventricular imbalance. In all these
instances, it is the precise atrioventricular
connections that determine the surgical
Left
atrium
Right
atrium
Dominant
left ventricle
Fig. 8.1 This specimen, prepared by slicing the heart in four-chamber

Base
Right
Left
Apex
projection, shows the essence of the double-inlet atrioventricular connection.

Note that the cavity of only one ventricle is visible. It possesses ne crisscrossing apical trabeculations, identifying it as a dominant left ventricle.
There is a second ventricle, albeit incomplete because it lacks its inlet
component, positioned anterosuperiorly, and thus is not sectioned in this cut.
In this heart, both atrioventricular junctions (black braces) are connected to
the dominant ventricle through separate atrioventricular valves.
246
Left
atrium
Right
atrium
Dominant
right ventricle
Base
Fig. 8.2 This specimen is also prepared by making a section in the fourLeft
Right
chamber plane. It shows a double inlet to a dominant right ventricle, as both

atrioventricular junctions (white braces) drain to the dominant ventricular
chamber (compare with Figure 8.1). Note, however, that there is a common
atrioventricular valve (white double-headed arrow). The section also
demonstrates the incomplete left ventricle, which lacks its inlet component.
Incomplete
left ventricle
Apex
Base
Left
atrium
Left
Right
Right
atrium
Apex
Dominant
right ventricle
Fig. 8.3 This specimen, windowed and viewed in anatomical

orientation, has a double inlet (white braces) to a dominant right
ventricle, but through two separate atrioventricular valves. The
inset, photographed from behind, shows that one papillary muscle
of the left atrioventricular valve (arrowed) retains its position
within the incomplete left ventricle.
approach. Whenever patients are found

with a double-inlet connection as dened
here, their hearts will perforce be
functionally univentricular. The chosen
surgical therapeutic option will, almost

certainly, be to create the Fontan
circulation although, rarely, it can be
possible to septate the dominant ventricle5.
It will be exceedingly rare for patients to be

encountered with a truly solitary ventricle.
Most usually, there will be two ventricles in
the setting of a double-inlet, one being
dominant and the other incomplete, the

smaller ventricle lacking at least the greater
part of its inlet component4.
Although the surgical options are the
same for all patients with the double-inlet
atrioventricular connection5, the hearts
themselves can show marked anatomical
variation. They can exist with any
arrangement of the atrial chambers, with
one of three ventricular morphologies, with
the variations in valvar morphology
discussed above, with any ventriculoarterial
connection, and with varied associated
malformations6. From the anatomical
stance, and probably from the stance of
long-term follow-up, the most important
differentiating feature is the morphology of
the dominant ventricle. There are three
possibilities7. The rst, and most common,
is the arrangement in which the atrial
chambers are connected to a dominant left
ventricle in the presence of an incomplete
right ventricle. The second group comprises
those with a dominant right and an
incomplete left ventricle. The nal small set
is made up of those patients having a solitary
ventricle of indeterminate morphology.
These three variants must be distinguished
from other hearts that, in essence, represent
huge ventricular septal defects.
A potential means of correcting these
hearts with a double inlet surgically is to
septate the dominant or solitary ventricle5.

The overall morphological arrangement,
however, usually conspires to defeat this
option. The most frequent surgical tactic,
therefore, is to use the Fontan procedure or
one of its modications810. We will
concentrate, therefore, on those anatomical
features inuencing the Fontan procedure,
although we will discuss the morphologies
that lend themselves to, or compromise,
septation.
In most instances, both atrioventricular
junctions are connected to a dominant left
ventricle, in the presence of an incomplete
morphologically right ventricle
(Figure 8.4). The chamber lacks its inlet
component, either completely when the
atrial chambers are exclusively connected
to the dominant left ventricle (Figures 8.1,
8.4, 8.5), or for the greater part when there
is overriding of one (Figure 8.6), or rarely
both, atrioventricular junctions. The
incomplete ventricle can be positioned to
the right (Figure 8.7) or the left
(Figure 8.8) of the dominant ventricle, but
it is always located anterosuperiorly, with
the atrioventricular connections opening
posteriorly to the hypoplastic ventricular
septum (Figures 8.4, 8.9). The morphology
of the incomplete ventricle is markedly
different when it supports the pulmonary
trunk (Figures 8.6, 8.10), the
ventriculoarterial connections being

concordant, compared to the more usual
arrangement with discordant
ventriculoarterial connections when the
aorta arises from the incomplete ventricle
(Figures 8.4, 8.7, 8.8). In all instances,
nonetheless, the incomplete chambers
possess an apical component of
morphologically right ventricular pattern.
They are separated from the dominant
ventricle by the hypoplastic muscular
ventricular septum (Figure 8.4), which
carries the atrioventricular conduction axis,
and which is nourished by septal
perforating arteries11.
The crucial feature in terms of surgical
correction is the dimensions of the
ventricular septal defect, as this must be of
adequate size to support the systemic
circulation irrespective of the precise
surgical tactics to be adopted. If it is
restrictive (Figure 8.4), it may need to be
surgically enlarged, although more
frequently the option is to by-pass the
restrictive area by opting for the Norwood
approach, or performing a DamusKaye
Stansel procedure. Should the surgeon opt
to enlarge the ventricular septal defect, the
major anatomical feature of concern is the
course of the axis of atrioventricular
conduction tissue12. Although always
originating from an anomalously located
Aorta
Incomplete
right ventricle
Pulm.
trunk
Dominant
left ventricle
Sup.
Left
Right
Inf.
247
Fig. 8.4 The reconstructed computed tomographic angiogram

shows, in exemplary fashion, the essential anatomy of a doubleinlet left ventricle. Both atrioventricular junctions (white arrows)
open into the dominant left ventricle, which gives rise to the
pulmonary (pulm.) trunk. The aorta arises from the incomplete
right ventricle, which is separated from the dominant ventricle by
the apical ventricular septum (star). The hole between the
ventricles (double-headed black arrow) is a ventricular septal
defect, which is restrictive in this example.
248
Base
Left
Right
Pulmonary trunk
Sup.
Right
Left
VSD
Apex
Inf.
Pulmonary
valve
Infundibulum
LAVV
Right
atrium
RAVV
Dominant
left ventricle
Fig. 8.5 This heart is an example of the most common form of

double-inlet left ventricle. The dominant left ventricle has been
opened in clam-shell-like fashion, and the specimen is shown in
anatomical orientation. The abnormal course of the conduction
axis has been superimposed on the picture as the red cross-hatched
area. LAVV and RAVV, left and right atrioventricular valves; VSD,
atrioventricular node (see later),

irrespective of its relationship relative to
the dominant left ventricle, the axis always
extends posteroinferior to the defect when
viewed from the incomplete right ventricle,
being carried on the left ventricular aspect
of the septal crest (Figure 8.5). When the
ventriculoarterial connections are
discordant, the incomplete right ventricle
usually has a very short outlet portion
(Figures 8.7, 8.8), making it difcult to
remove any tissue cephalad to the defect.
The safest way to enlarge the defect
surgically, therefore, is to remove a wedge
of the muscular ventricular septum closest
to the obtuse margin of the heart
(Figure 8.11)12.
Straddling tension apparatus
Fig. 8.6 In this specimen with a double-inlet left ventricle, the

incomplete right ventricle is photographed from the front with the
heart positioned anatomically. The ventriculoarterial connections
are concordant, with the pulmonary trunk seen supported by an
infundibulum and arising from the incomplete right ventricle. The
right atrioventricular valve straddles and overrides, but its greater
part remains connected within the dominant left ventricle, so the
connection remains one of a double inlet.
In a small proportion of patients

with a double-inlet left ventricle, the
ventriculoarterial connections may be
concordant7, or there can be a double outlet
from the dominant left or incomplete right
ventricle. The variant with concordant
ventriculoarterial connections is of
signicant anatomical interest. The lesion
is described eponymously as the Holmes
heart13. In this setting, the incomplete right
ventricle, when seen in isolation, can be
virtually indistinguishable from the
incomplete right ventricle seen in hearts
with tricuspid atresia (compare Figures 8.9
and 8.12). The ventricular septal defect is
often restrictive in this form of double-inlet
left ventricle (Figure 8.9), and the same
rules pertain should it require surgical

enlargement. This is unlikely to be
performed, however, unless a septation is
also attempted. The more likely approach
will be to perform a Fontan procedure. The
incomplete right ventricle will then be
excluded from the circulation.
Taken overall, the Fontan procedure, or
one of its modications, will be the likely
operation of choice for most patients with a
double-inlet left ventricle. It can be used
successfully for all the various anatomical
variants, providing that the haemodynamic
criterions are satisfactory14. Almost always,
the procedure includes connecting the
systemic venous returns, in one way or
another, to the pulmonary arteries. This is
249
VSD
Aorta
Aorta
VSD
Base
Base
Right
Left
Right
Left
Apex
Apex
Fig. 8.7 In this specimen with a double-inlet left ventricle and
Fig. 8.8 In this specimen with a double-inlet left ventricle, again
discordant ventriculoarterial connections, viewed anatomically,

the incomplete right ventricle, supporting the aorta, is right-sided.
VSD, ventricular septal defect.
with discordant ventriculoarterial connections, and viewed

anatomically, the incomplete rudimentary right ventricle,
supporting the aorta, is left-sided. VSD, ventricular septal defect.
Outlet septum
Apical septum
Aorta
RAVV
Dominant LV
LAVV
Fig. 8.9 This specimen with a double-inlet left ventricle and

Sup.
Apex
To pulmonary trunk
Base
Inf.
discordant ventriculoarterial connections has been sectioned to

replicate the parasternal long axis view, and is photographed in
anatomical orientation. Note that the apical ventricular septum is
positioned between the apical components of the dominant left
and incomplete right ventricles. This septum is the hypoplastic
muscular ventricular septum, as it carries the conduction axis, and is
nourished by the septal perforating arteries. LV, left ventricle;
RAVV and LAVV, right and left atrioventricular valves.
250
Pulm. trunk
Long
infundibulum
Aorta
VSD
Fig. 8.10 In this specimen, again with a double-inlet left ventricle,

Sup.
Left
Right
Inf.
Apical RV trabeculations
but with concordant ventriculoarterial connections, as with the

heart shown in Figure 8.6, the right-sided incomplete right
ventricle (RV) supports the pulmonary (pulm.) trunk, with the aorta
arising from the dominant left ventricle. This is the so-called
Holmes Heart. Note the length of the infundibulum of the
incomplete ventricle when it supports the pulmonary trunk,
compared to those supporting the aorta (see Figures 8.7 and 8.8).
Restrictive VSD
Aorta
Fig. 8.11 This specimen, photographed in surgical orientation,
Left
Inf.
Sup.
Right
usually achieved now by either creating a

conduit within the right atrium to produce
a so-called total cavopulmonary
connection15, or inserting an extracardiac
conduit that connects the inferior caval
shows the relationship of the axis of the atrioventricular

conduction tissue to the ventricular septal defect in a double-inlet
left ventricle with discordant ventriculoarterial connections when
viewed from the incomplete right ventricle. The ventricular septal
defect (VSD) is restrictive. The course of the conduction axis is
superimposed in red, with the site of the anomalous
atrioventricular node shown by the star, while the cross-hatched
oval shows the area that can be removed without inicting trauma
to the conduction tissues.
vein to the pulmonary arteries, combining

this with a Glenn procedure16. In the past,
the roof of the right atrium at its junction
with the appendage was often connected
directly to the pulmonary trunk. Such an
atriopulmonary connection still remains a

surgical option. When there is
juxtaposition of the atrial appendages, a not
infrequent associated malformation with a
double-inlet left ventricle, this anastomosis
Pulm. trunk
251
Long
infundibulum
Aorta
Right atrium
Fig. 8.12 This specimen has classical tricuspid atresia with
Sup.
Sup.
VSD
Left
Right
Inf.
Apical RV trabeculations
concordant ventriculoarterial connections, and is photographed in

anatomical orientation. Note the similarity in the morphology of
the incomplete right ventricle (RV) in this setting with its long
infundibulum, with that seen in a double-inlet left ventricle when
the ventriculoarterial connections are concordant (see Figure 8.9).
Pulm., pulmonary; VSD, ventricular septal defect.

Aorta
Superior caval vein
Left
Fig. 8.13 In this heart, photographed in the operating room, the

Inf.
Sup.
Sinus node
Right
becomes even simpler. If the atrium is to be

connected to the pulmonary arteries, either
directly or by construction of an internal
conduit, it is crucial for the surgeon to
avoid the sinus node and its arterial supply.
In this respect, the key area is the superior
interatrial groove, through which courses
artery to the sinus node, arising from the right coronary artery, is
seen coursing through the interatrial groove to cross the cavoatrial
junction at the crest of the right atrial appendage. The sinus node is
visible as a pale area within the terminal groove.
the artery to the sinus node irrespective of

its arterial origin (Figure 8.13). Use of an
internal or external conduit obviously
obviates the need to isolate the systemic
venous return from the right
atrioventricular valve surgically, an added
manoeuvre that is essential if attempting an
atriopulmonary connection. If this has to be

done, there are at least two options open to
the surgeon. It can be done by securing a
patch across the vestibule of the valve. It
can also be achieved by deviating the atrial
septum to the parietal border of the right
atrium, thus leaving both valves draining to
252
the dominant ventricle. With both of these

options, it is important to avoid the
atrioventricular node, which in a doubleinlet left ventricle is no longer located at the
apex of the triangle of Koch17. This is
because the muscular ventricular septum,
carrying the ventricular conduction tissues,
does not reach the crux of the heart, rising
instead to the acute margin of the
ventricular mass (Figure 8.5). The
atrioventricular node, therefore, is located
within the quadrant of the junction related
to the mouth of the right atrial appendage
(Figure 8.14). This area is the major site of
danger. Irrespective of its precise location,
nonetheless, the node can be avoided by
securing the patch at least one centimetre
above the attachments of the leaets of the
right atrioventricular valve (Figure 8.14).
The features discussed above, however,
are less pertinent to the modern-day
surgeon, as it is now the rule to insert an
extracardiac conduit so as to direct the
inferior caval venous blood to the lungs or
to the superior caval vein. Like an internal
bafe, this procedure produces the total
cavopulmonary connection16 with the
extracardiac conduit also having
advantages in terms of the pattern of ow
into the pulmonary arteries. Should it be
decided to construct an internal bafe, the
major areas to be avoided are again the
dictate the insertion of a conduit from the

dominant ventricle to the pulmonary
arteries. Remarkably few patients will
match all these criterions, and become
suitable candidates for septation. In those
that are, the major consideration during
surgery will be to avoid the axis of
atrioventricular conduction tissue, thus
avoiding iatrogenic heart block. It is also
necessary, of course, to preserve the sinus
node and its arterial supply (Figure 8.13).
The atrioventricular conduction axis in
hearts with a double-inlet left ventricle
takes its origin from an anomalous node
located anterocephalad within the right
atrioventricular junction, beneath the
mouth of the atrial appendage
(Figure 8.14). From this anterior node, the
atrioventricular bundle penetrates the
lateral end of the area of brous continuity
between the leaets of the pulmonary and
the right atrioventricular valves. When the
incomplete right ventricle is left-sided, as it
will be in most cases suitable for septation,
the non-branching atrioventricular bundle
encircles the anterior quadrants of the
pulmonary orice to reach the rightwards
margin of the muscular ventricular septum
(Figure 8.5). The left bundle branch then
cascades down the left ventricular aspect of
the septum, the axis of conduction tissue
itself being located anterocephalad to the
Morphologically left ventricle
Apex
Sup.
sinus node and its arterial supply. Such

problems are avoided when inserting an
extracardiac conduit.
Very few surgeons now choose septation
for patients with a double-inlet left
ventricle, even though it is perhaps the
most logical surgical option5. Relatively few
patients, however, have anatomy suitable
for successful septation. Both
atrioventricular valves must be competent
structures, and the relationships of
dominant and incomplete ventricles must
permit the construction of a patch to
channel ow from the left atrioventricular
valve to the ventricular septal defect. This
means that the incomplete right ventricle
should be left-sided (Figures 8.4, 8.8) or, at
worst, directly anterior. The dominant
ventricle also needs to be of good size,
which means that septation is unlikely to be
attempted in infancy. If the pulmonary
circulation is not protected by naturally
occurring stenosis, this must be achieved
immediately after birth by banding the
pulmonary trunk. Banding itself tends to
promote hypertrophy of the ventricular
myocardium, and narrowing of the
ventricular septal defect. It may also be
necessary, therefore, to combine
enlargement of the defect (Figure 8.11)
with septation. The presence of naturally
occurring subpulmonary stenosis may
Inf.
Site of suture line

within right atrium
Base
Fig. 8.14 This cartoon, drawn in surgical orientation, shows the

Connecting node not
in triangle of Koch
danger area of the right atrioventricular orice (red double-headed

arrow) in hearts with a double-inlet left ventricle, and the way the
conduction tissue can be avoided by suturing above the level of the
atrioventricular junction (blue dashed line).
ventricular septal defect when viewed

either through a sh-mouth incision in the
dominant left ventricle (Figure 8.15), or
through the right atrioventricular valve
(Figure 8.16). Unless a conduit is also
placed from the dominant left ventricle to
the pulmonary arteries, the line of sutures
used to septate the dominant ventricle must
cross this course of the axis of conduction
tissues (Figure 8.15). The axis itself is
relatively narrow as it emerges from the

area of brous continuity between the
pulmonary and right atrioventricular
valves. If this site is known with precision,
the sutures can be placed so as to avoid the
axis. All of these considerations, taken
together, indicate that septation is a
formidable surgical procedure if the
pathways through the dominant ventricle
are to be separated without insertion of a
LAVV
RAVV
Conduction tissue
Line of suture for complete septation
Line of suture for double-outlet left
ventricle
253
conduit, or involuntary induction of

atrioventricular dissociation. Septation can
be a potentially feasible option for patients
with a double-inlet left ventricle and
concordant ventriculoarterial connections.
If attempted, the same rules hold good for
the disposition of the axis of conduction
tissue, but a potential caveat is that the
ventricular septal defect is most frequently
restrictive or obstructed (Figures 8.4, 8.9).
Fig. 8.15 The cartoon shows the dominant left ventricle with a
double inlet as seen through a sh-mouth incision in the apex of
the ventricle. The conduction axis is marked in green (see also
Figure 8.5). The potential line for complete septation, placing the
pulmonary trunk in communication with the systemic venous
return (blue dashed line), crosses the conduction axis. To avoid the
conduction axis (green dashed line), it is necessary to deviate the
site of septation so that both outow tracts remain in
communication with the pulmonary venous return through the left
atrioventricular valve. The star shows the location of the ventricular
septal defect. LAVV and RAVV, left and right atrioventricular
valves.
Branching bundle
Penetrating bundle
Atrioventricular ring tissue
Transitional cell zone
Fig. 8.16 This cartoon, again drawn in surgical orientation, shows
Pulmonary valve
the view of the dominant left ventricle in the setting of a doubleinlet left ventricle with discordant ventriculoarterial connections,
and the course of the conduction axis, in green, as it would be seen
by the surgeon working through the right atrioventricular valve.
254
Subaortic outlet
Subpulmonary outlet
LAVV
RAVV
Sup.
right ventricle is photographed in anatomical orientation. Both

atrioventricular valves connect exclusively to the dominant right
ventricle, while the extensive outlet septum (white double-headed
arrow) separates the origins of the two arterial trunks. RAVV and
LAVV, right and left atrioventricular valves.
Left
Right
Fig. 8.17 This specimen with a double-inlet and double-outlet
Inf.
The other variants of the double-inlet

ventricle are much more rare, and hardly
ever are suitable for septation. The doubleinlet right ventricle (Figure 8.17) is seen
most frequently with a double outlet from
the dominant right ventricle, often with
straddling and overriding of the left
atrioventricular valve (Figure 8.3)18. The
incomplete left ventricle, usually found in
the left-sided position, although sometimes
found to the right, is nothing more than a
pouch with left ventricular apical
trabeculations (Figure 8.18). It is always
found posteroinferiorly, and is dorsal to the
atrioventricular valves, in the
diaphragmatic surface of the ventricular
mass. The Fontan procedure is the most
likely therapeutic surgical option, in which
case the landmarks within the atrial
chambers, together with the surgical
caveats, are as discussed for the doubleinlet left ventricle. Sometimes, a doubleinlet right ventricle can be found with
concordant ventriculoarterial connections
(Figure 8.19), usually with a common valve
guarding the atrioventricular junctions
(Figures 8.3, 8.20). The axis of
atrioventricular conduction in this setting
descends from a node located within its
usual position in the triangle of Koch. The

exception is when the incomplete left
ventricle is right-sided, the conduction axis
arising from an anomalous node19, as in
congenitally corrected transposition (see
later).
Those rare hearts with a double inlet to a
solitary and indeterminate ventricle usually
coexist with isomerism of the atrial
appendages, when the multiple associated
malformations, particularly the anomalous
venoatrial connections, dominate the
picture. Rare examples can be found with
the usual arrangement of the atrial
appendages. In this setting, the crossing of
the tension apparatus from the
atrioventricular valves, along with the
particularly coarse indeterminate apical
trabeculations, tend to conspire against
septation (Figure 8.21). Patients with
solitary ventricles, therefore, are also most
likely to undergo repair by means of the
Fontan option, and the same rules apply as
discussed earlier.
Hearts with huge ventricular septal
defects (Figure 8.22) can sometimes be
confused with a double inlet to a solitary
and indeterminate ventricle. Even in those
hearts with huge septal defects, septation
remains a formidable undertaking. In these

rare patients, it is the persisting rim of
muscular septum separating the apical
trabecular components, together with the
inlet septum rising to the crux, that provide
the anatomical landmarks. The axis of
conduction tissue descends from the
regular atrioventricular node when the
atrioventricular connections are
concordant in this setting (Figure 8.22).
TRICUSPID AND MITRAL ATRESIA

In most instances, tricuspid atresia is
produced by complete absence of the right
atrioventricular connection (Figure 8.23).
Many hearts with mitral atresia exist
because of the absence of the left
atrioventricular connection (Figure 8.24).
In these hearts, because of the absence of
one of the atrioventricular connections, and
hence with only one of the atrioventricular
junctions making contact with the
ventricular mass, the ventricular
morphology is comparable to that seen in
those with a double inlet (see Figure 8.2).
All three atrioventricular connections are
univentricular. The solitary
atrioventricular valve, nonetheless, can
Sup.
255
Left atrioventricular valve

Left atrium
Post.
Ant.
Inf.
Fig. 8.18 This picture, again viewed in anatomical orientation,
shows the location of the left-sided incomplete left ventricle in the

heart shown in Figure 8.17 with a double-inlet right ventricle. The
incomplete ventricle is no more than an outpouching from the
dominant ventricle. The black double-headed arrow shows the
hypoplastic apical ventricular septum.
Common AVV to RV
Aorta
Incomplete
left ventricle
Sup.
Post.
Ant.
Inf.
Fig. 8.19 In this picture, again in anatomical orientation, an

example is seen of a double-inlet right ventricle in which the
incomplete left ventricle gives rise to the aorta. There is a common
atrioventricular valve (AVV) joining both atriums to the dominant
right ventricle (RV), shown in cross-section in Figure 8.2.
256
Sup.
Right
Pulm. trunk
Left
Inf.
Dominant
right ventricle
Fig. 8.20 This is another picture of the dominant right ventricle

from the heart shown in Figure 8.19. Both atriums drain to the
dominant ventricle through the common atrioventricular (AV)
valve. The ventriculoarterial connections are concordant, with the
pulmonary (pulm.) trunk arising from the dominant ventricle.
Common AV valve
Subpulmonary outlet
Coarse apical trabeculations
Left
AV valve
Fig. 8.21 This specimen, seen in anatomical

Right
AV valve
Sup.
Left
Right
Subaortic outlet
Inf.
straddle and override (Figure 8.25). This

produces a uniatrial, but biventricular,
atrioventricular connection20.
Although the variations in terms of
ventricular morphology when one
atrioventricular connection is absent are
the same as for the double-inlet

ventricle, most examples fall into what
can be considered to represent classical
types of atrioventricular valvar atresia.
We will concentrate our attention,
therefore, on these anticipated variants,
orientation, with the ventricle opened in

clam-shell-like fashion, has a double inlet to
and double outlet from a solitary ventricle of
indeterminate morphology, which has very
coarse apical trabeculations. Note that the
tension apparatus of both atrioventricular (AV)
valves has a common origin, making septation
exceedingly difcult, if not impossible.
while drawing attention to the less

familiar formats.
In tricuspid atresia, it is the
morphologically right atrium that is blindending, usually with no vestige of the
tricuspid valve in its oor (Figure 8.26).
Left AV valve
Aorta
Sup.
257
Post.
Ant.
Inf.
RAVV
Fig. 8.22 This view, in anatomical orientation, shows the left
Left ventricular
apical trabeculations
ventricle in a heart with concordant atrioventricular connections

and a huge ventricular septal defect. The site of the axis of
atrioventricular conduction tissue has been marked with a red
dotted line on the left ventricular aspect of the hypoplastic
ventricular septum, which runs to the apex (star), where it separates
the apical ventricular components. The right atrioventricular valve
(RAVV) is connected within the right ventricle, hence the
concordant nature of the atrioventricular connections.
Left-sided inlet
Left atrium
Right atrium
Dominant LV
Fig. 8.23 The heart has been sectioned to replicate the fourchamber echocardiographic view. It shows complete absence of the
right atrioventricular (AV) connection, the atrioventricular groove
being lled with adipose tissue, and containing the right coronary
artery. This is the essence of the usual variant of tricuspid atresia.
The section passes mostly through the dominant left ventricle (LV)
(compare with Figure 8.1), but the posterior part of the incomplete
right ventricle is just visible (star).
Absent AV connection
Because of the absent connection, the right

ventricle is incomplete, lacking its inlet
component21. The apical trabecular part of
the incomplete ventricle, as with the double-
inlet left ventricle (Figures 8.4, 8.10), is

separated from the dominant ventricle by the
apical muscular ventricular septum
(Figure 8.27). As in the double-inlet left
ventricle, the apical ventricular septum does

not extend to the crux, but rather extends to
the acute margin of the ventricular mass
(Figure 8.28). Surgical palliation involves the
258
Absent left
AV connection
Left atrium
Slit-like
left ventricle
Right atrium
Fig. 8.24 This heart with classical mitral

atresia, from a patient with hypoplasia of the

left heart, has been sectioned to replicate the
four-chamber echocardiographic view
(compare with Figure 8.23). There is complete
absence of the left atrioventricular (AV)
connection. The section has also transected the
ventricular septum, showing the site of the
posteroinferior and slit-like incomplete left
ventricle (compare with Figure 8.2).
Sup.
Left
Right
Inf.
Sup.
Right
Left atrium
Left
Inf.
Right atrium
Dominant LV
Incomplete
right ventricle
Fig. 8.25 This heart, with the absence of the right atrioventricular
connection (white dotted lines), is sectioned to simulate the
echocardiographic four-chamber view. The solitary atrioventricular
junction (white double-headed arrow) is shared between the
dominant left (LV) and the incomplete right ventricles because of
straddling of the solitary atrioventricular valve (arrow head). This
arrangement produces a uniatrial but biventricular connection.
Superior caval vein
Dimple
Sup.
Post.
Ant.
Inf.
Fig. 8.26 This view of the opened right atrium from a specimen
Oval fossa
Pectinate muscles
Coronary sinus
with tricuspid atresia, seen in anatomical orientation, shows the

muscular oor due to complete absence of the right
atrioventricular connection. The site of the atrioventricular node
has been superimposed in green, and is closely related to the
dimple.
Sup.
Right
Left atrium
Left
Inf.
Right atrium
Fig. 8.27 This specimen with classical tricuspid atresia due to

Fontan operation, or one of its modications.

In hearts with concordant ventriculoarterial
connections, attempts were often made
during the early period of evolution of the
Fontan procedure to incorporate the
absence of the right atrioventricular connection (white dashed

lines) and concordant ventriculoarterial connections, has been
sectioned in a parasternal long axis fashion, and is photographed
positioned on its apex. The hypoplastic ventricular septum
separates the apical parts of the dominant left and incomplete
right ventricles (compare with Figure 8.10). The communication
between the ventricles is a ventricular septal defect (star).
incomplete right ventricle into the

pulmonary circulation22. Presently, most
surgeons have shifted to constructing total
cavopulmonary connections, now mostly
using external conduits. If the conduit is to
be constructed within the right atrium, the

architecture of its muscular walls, and the
arterial supply, should be disturbed as little
as possible. Preservation of the sinus node
and its blood supply is particularly
259
260
Ant. intervent. artery
Aorta
VSD
Fig. 8.28 This dissection of a specimen with tricuspid atresia with

Sup.
Left
Right
Acute marginal artery

Inf.
important, as atrial arrhythmia is a

recognised life-threatening postoperative
complication, particularly in the long term.
The rules for avoidance of these structures
are as described earlier for the double-inlet
ventricle (Figure 8.9). Whenever a wellformed Eustachian valve is encountered, it
should be preserved. Such a valve can be
incorporated in an internal bafe. Creation of
such an internal bafe requires the surgeon
to make a direct and wide connection
between the right atrium and the pulmonary
arteries. In such circumstances, the artery to
the sinus node can be at risk as it traverses
the interatrial groove. This potential
complication is avoided when inserting an
extracardiac conduit.
When the Fontan procedure is performed
in hearts with discordant ventriculoarterial
connections, it may be necessary to resect the
margins of the ventricular septal defect
should it be restrictive. This must be done in
a way that avoids the atrioventricular
conduction axis on the left ventricular aspect
of the septum12. When seen from the right
ventricle, this runs posteroinferior to the
septal defect as in the double-inlet left
ventricle (Figure 8.29). The rare exception is
when the typical muscular ventricular septal
defect is atretic, and an apical trabecular
concordant ventriculoarterial connections, viewed in anatomical

orientation, shows that the right delimiting coronary artery
descends at the acute margin of the ventricular mass from the right
coronary artery, rather than the crux. The other delimiting artery is
the anterior interventricular (ant. intervent.) artery. Note the
anterosuperior location of the incomplete right ventricle. VSD,
defect provides the interventricular

communication (Figure 8.29).
In very rare cases, tricuspid atresia may
be produced because an imperforate valvar
membrane is interposed between the right
atrium and ventricle. This can be seen as
part of pulmonary atresia with an intact
ventricular septum (Figure 8.30). An
imperforate valve can also be found when
there are concordant atrioventricular
connections and a ventricular septal defect
(Figure 8.31), albeit very rarely, or as part
of Ebsteins malformation (Figure 8.32)23.
We are unaware of any patient in whom the
junction blocked by the imperforate
membrane has been of sufcient size to
permit resection of the valve, and
replacement with a valvar prosthesis.
Operative treatment of mitral atresia is
complicated because of its usual association
with aortic atresia. This, of course, is one of
the typical combinations producing
hypoplasia of the left heart24. Direct
surgical correction involves reconstruction
of the arterial pathways as an initial
procedure, employing the Norwood
protocol25 or one of its modications. This
is followed by a subsequent modied
Fontan procedure. The options in these
procedures are dictated by the anatomy of
the aortic pathways rather than the

ventricular morphology, with the key
features being the relations of the arterial
duct (Figures 8.33, 8.34), and the size of
the aortic root (Figures 8.358.38). In some
patients having atresia of the left
atrioventricular valve, however, the aortic
outow tract is patent. Mitral atresia may
not be the best term for description of all
these patients. This is because, in many,
the morphology suggests that, had the left
atrioventricular connection been formed, it
would have been guarded by a
morphologically tricuspid valve. The right
atrium in this setting is connected to a
morphologically left ventricle, with the
incomplete right ventricle being anterior
and left-sided, usually with discordant
ventriculoarterial connections
(Figure 8.39). In this setting, there is no
direct route for the pulmonary venous
return to reach the dominant left ventricle.
The blood must cross an atrial septal defect
and traverse the right-sided
atrioventricular valve. Initial survival in
these cases, therefore, depends on the state
of the atrial septum. Should the septum be
restrictive, it will need to be enlarged.
Some sort of modied Fontan procedure
will then be the nal option, usually a
261
Regular muscular defect
Sup.
Apical muscular defect

Right
Left
Inf.
Right
atrium
Right atrium
Imperforate
tricuspid valve
Apex
Inf.
Sup.
Base
Hypoplastic right ventricular inlet
Fig. 8.30 This specimen is sectioned across the right atrioventricular

junction. There is pulmonary atresia with an intact ventricular septum, with
mural hypertrophy obliterating the apical (star) and outlet ventricular
components. In addition, the tricuspid valve is imperforate. The
atrioventricular connections are concordant.
Fig. 8.29 The cartoon, drawn in surgical orientation, shows

the route of the axis of the atrioventricular conduction tissue,
illustrated in green, relative to the usual ventricular septal
defect in tricuspid atresia, and to a defect opening between the
apical trabecular component of the incomplete right ventricle
and the dominant ventricle. In the latter situation, the axis is
superior relative to the defect.
cavopulmonary connection. As with

tricuspid atresia, left-sided atresia can be
produced by an imperforate
atrioventricular valve rather than being due
to the absence of the left atrioventricular
connection. The imperforate valve can be
found with biventricular (Figure 8.40) or
double-inlet (Figure 8.41) atrioventricular
connections. Irrespective of the specic
morphology, the only surgical option will
be to create a functionally univentricular
connection, the Fontan circulation being
constructed following one of the options
discussed earlier.
TRANSPOSITION
A particularly important lesion is
represented by the combination of
concordant atrioventricular with
discordant ventriculoarterial connections.

Most clinicians refer to the combination
simply as transposition. This usage,
although widespread, is less than precise.
In the past, disagreements were frequent as
to whether the term transposition should
be used only to describe hearts with
discordant ventriculoarterial
connections26, or for description of any
heart with an anterior aorta27. More
signicantly, when transposition is used to
describe discordant ventriculoarterial
connections, the term is not restricted to
the combination with concordant
Transposition, if dened simply on the
basis of discordant ventriculoarterial
connections, can also coexist with
discordant atrioventricular connections,
with a double-inlet ventricle, with an
absence of one atrioventricular connection,
or with isomerism and biventricular but

mixed atrioventricular connections. In all
these circumstances, it is the arrangements
at the atrioventricular junctions that
become the dominant features. There is,
therefore, a need for a term that describes
only the combinations of concordant
atrioventricular and discordant
(Figure 8.42). Previously, our preferred
term had been complete transposition. We
now recognise that this term itself is less
than perfect. Complete, as an adjective, was
rst used to indicate that both arterial
trunks were transposed across the
ventricular septum, thus distinguishing the
arrangement from the presumed partial
arrangement, in which only the aorta was
transposed across the septum. The latter
anomaly is universally described now as a
double-outlet right ventricle, although as
262
Sup.
Right
Left
Inf.
Fig. 8.31 The right atrium in this specimen is viewed from above
Imperforate tricuspid valve
Eustachian valve
and behind, in anatomical orientation, to show the atrial aspect of

an imperforate tricuspid valve. In this heart, the atrioventricular
connections were concordant (compare with Figure 8.26), and
there was a ventricular septal defect.
Imperforate Ebsteins malformation
Right
atrium
Sup.
Right
Left
Atrialised right
ventricular inlet
Inf.
we will discuss, problems also remain in

dening this entity. This does not alter the
fact that, in the strictest sense, all examples
of discordant ventriculoarterial
connections represent complete
Fig. 8.32 In this heart with an imperforate tricuspid valve and

concordant atrioventricular connections, the tricuspid valve itself is
deformed by Ebsteins malformation. The dotted green line shows
the location of the right atrioventricular junction.
transposition28. We suggest, therefore, that

the combination of discordant
ventriculoarterial with concordant
atrioventricular connections be considered
the default option for use of the term
transposition. If discordant ventriculoarterial

connections are found in other settings, we
describe them as congenitally corrected
transposition, or tricuspid atresia with
transposition, and so on.
Transverse aortic arch
263
Ascending aorta
Pulmonary
trunk
Sup.
Fig. 8.33 This specimen, viewed from the left side in anatomical
orientation, shows the typical arrangement of the arterial trunks in
hypoplastic left heart syndrome due to combined aortic and mitral
atresia, but with the subclavian artery arising distally to the arterial
duct. Note the grossly hypoplastic transverse aortic arch.
Post.
Ant.
Inf.
Arterial duct

Pulmonary
trunk
Arterial duct
Fig. 8.34 This computed tomographic angiogram, viewed from
Sup.
Post.
Ant.
Inf.
It is less than appropriate, however, to

use d-transposition as the same default
option. This latter term fails to account
accurately for all patients having the usual
atrial arrangement with concordant
the left side in anatomical orientation (compare with Figure 8.33),

shows the typical arrangement of the arterial trunks in hypoplastic
left heart syndrome, but with the subclavian artery arising in its
normal position proximal to the arterial duct. Note the major ow
pathway through the arterial duct to the descending aorta.
atrioventricular and discordant

ventriculoarterial connections. An
important subset of these patients can
have a left-sided aorta (Figure 8.43). Nor
does d-transposition describe accurately
the majority of patients with the

combinations of mirror-imaged atrial
arrangement, concordant atrioventricular,
and discordant ventriculoarterial
connections (Figure 8.44). All these
264
Sup.
Left
Right
Arterial duct
Inf.
Pulmonary
trunk
Good-sized
ascending aorta

the front, shows hypoplastic left heart syndrome with combined
aortic and mitral atresia. In this heart, the aorta is of good size,
despite the gross hypoplasia of the left ventricle.
Pulmonary
trunk
Sup.
Fig. 8.36 The computed tomographic angiogram from the patient
Ant.
Post.
Inf.
patients, nonetheless, can be described

accurately as having transposition.
However, discordant ventriculoarterial
connections, as we have already
Good-sized
ascending aorta
shown in Figure 8.34, viewed from the right side, shows arterial
pathways that are directly comparable to those seen in the heart
dissected to produce Figure 8.35.
emphasised, can also occur in the setting

of isomerism of the atrial appendages.
The anomalies at the venoatrial and
atrioventricular junctions then
dominate the anatomical and clinical

considerations. We exclude these cases,
therefore, from the category of
transposition as described here.
265
Arterial duct
Sup.
Left
Right
Inf.
Diminutive
ascending aorta
Pulmonary
trunk

the front, shows the arrangement of the arterial trunks in
hypoplastic left heart syndrome due to combined aortic and mitral
atresia when there is a diminutive ascending aorta.
Diminutive
ascending aorta
Sup.
Descending
aorta
Post.
Ant.
Inf.
Aortic isthmus
From the surgical standpoint, the

two major subgroups of hearts with
transposition are those without any
major additional complicating lesions,
usually described as simple transposition,
Fig. 8.38 The computed tomographic angiogram shows an

arrangement of the arterial pathways in hyoplastic left heart
syndrome directly comparable to that in the specimen illustrated in
Figure 8.37 with a diminutive ascending aorta. Note the shelf-like
coarctation lesion (arrow) at the origin of the aortic isthmus. The
shelf is composed of ductal tissue.
and those with additional malformations

sufciently severe to complicate the
clinical picture. The morphological
aspects of the complicating lesions will
be dealt with in turn, but the atrial
anatomy is comparable within the whole

group.
Now, corrective surgical procedures are
almost always performed at the arterial
level. Operations designed to redirect
266
Sup.
Ant.
Post.
Aorta
Inf.
Fig. 8.39 As shown by this specimen, in anatomical orientation
Absent left atrioventricular connection
Blind-ending left atrium
viewed from the left, mitral atresia is not always part of hypoplasia
of the left heart. In this example, there is absence of the left
atrioventricular connection, but the right atrium is connected to a
dominant ventricle with left ventricular trabeculations (not seen).
The incomplete right ventricle, supporting the aorta, is left-sided
and anterior. Had the left atrioventricular valve been formed, it
would almost certainly have been of tricuspid morphology.
Sup.
Post.
Ant.
Inf.
Imperforate
mitral valve
Hypoplastic left ventricle
venous blood at the atrial level should not

be forgotten, however, particularly as these
manoeuvres are an integral part of double
switch procedures. When planning these
operations, known as the Mustard and
Senning options, it is the disposition of the
cardiac nodes and their blood supply that is
Fig. 8.40 In this specimen, seen in anatomical orientation and

photographed from the left side, there is an imperforate mitral
valve in the setting of concordant atrioventricular connections.
Note the hypoplastic left ventricle and ventricular septal defect.
probably the most important factor. The

presence of discordant ventriculoarterial
connections does not affect the position of
the sinus node in any way, so the rules
enunciated in Chapter 2 for avoidance of
this node, and discussed earlier in the
setting of the Fontan procedure, are
equally applicable in the setting of

transposition. The entire terminal
groove should be avoided, as should the
crest of the atrial appendage, because the
node can extend in horseshoe fashion
across the crest, although it is usually in a
lateral position. The nodal artery may
267
Imperforate LAVV
RAVV

Sup.
Fig. 8.41 In this specimen, seen in anatomical orientation with

Right
the dominant left ventricle having been opened from the front,
there is a double inlet to the dominant ventricle, with the
incomplete right ventricle in the left-sided position, but with an
imperforate left atrioventricular valve (LAVV). The right
atrioventricular valve (RAVV) is patent.
Left
Inf.
Pulm.
trunk
Aorta
Morph.
left atrium
Pulm..
trunk
Morph.
left atrium
Aorta
Morph.
right atrium
Morph.
right atrium
Morph.
left ventricle
Morph.
right ventricle
Fig. 8.42 The cartoon shows the
Morph.
left ventricle
Mirror-imaged atrial arrangement
segmental combinations of
concordant atrioventricular and
discordant ventriculoarterial
connections that produce the
lesion usually described simply as
transposition. The arrangement
can exist in usual and mirrorimaged variants. Morph.,
morphologically; Pulm.,
pulmonary.
running across the superior border of the

oval fossa, usually described as being the
septum secundum (Figure 8.45). It is at
risk in this position when incising the
septum for either a Senning or Mustard
procedure, or during a BlalockHanlon
septostomy. A lateral course across the

atrial appendage is also signicant
(Figure 8.46).
Other considerations remain signicant
when carrying out a venous switch
procedure. Cannulation of the superior
Morph.
right ventricle
Usual atrial arrangement
enter the groove across the crest of the

appendage, or after it has taken a retrocaval
course. Of more importance is the course
taken by the nodal artery as is ascends the
interatrial groove. The artery frequently
burrows into the atrial musculature,
268
Anterior intervent.
coronary artery
Aorta
Fig. 8.43 This surgical view, taken through a median sternotomy,
Pulmonary
trunk
Left
Inf.
Sup.
Right
Morph. right
atrial appendage
Left-sided aorta
Fig. 8.44 In this heart with concordant atrioventricular and
Sup.
Left
Right
Inf.
shows a patient with transposition, dened on the basis of

concordant atrioventricular and discordant ventriculoarterial
connections, in which the aorta is anterior and leftward relative to
the pulmonary trunk. It would be a mistake to describe this
arrangement as d-transposition. Note the origin of the anterior
interventricular (intervent.) artery from the right coronary artery,
with passage across the subaortic infundibulum.
Morph.
right ventricle
discordant ventriculoarterial connections, shown in anatomical

orientation from the front, the aorta is left-sided relative to the
pulmonary trunk. In this case, the heart is in the right chest, with
the apex pointing to the right, in the setting of mirror-imaged atrial
arrangement. Although exhibiting l-transposition, the
arrangement is not congenitally corrected. Morph.,
morphologically.
269
Aorta
SCV
Oval fossa
Tricuspid
valve
Sup.
Fig. 8.45 This dissection of a specimen of transposition seen in
Ant.
Post.
anatomical orientation from the right side shows the relationship

of the artery to the sinus node, in this heart arising from the right
coronary artery, to the superior rim of the oval fossa. SCV, superior
caval vein.
Inf.
Sup.
Ant.
Post.
Inf.
Incision
Fig. 8.46 This anatomical specimen with transposition is

photographed in anatomical orientation from the right side to
show how the artery to the sinus node, with the site of the node
shown by the blue textured oval, when arising laterally from the
right coronary artery, has been transected by a standard incision in
the right atrial appendage.
caval vein should be performed a good

distance from the cavoatrial junction,
incising the right atrium well clear of the
terminal groove. Traction, suction, or
suturing should be avoided in the area of

the superior border of the groove. If an
incision is required across the terminal
groove to widen the newly constructed
pulmonary venous atrium in Mustards

operation, it can be made between the right
pulmonary veins without fear of damaging
the sinus node or its artery.
270
Sup.
Ant.
Post.
SCV
Inf.
Oval fossa
Fig. 8.47 This dissection is made in a normal heart, orientated
Terminal crest
Eustachian valve
Coronary sinus
This discussion is also pertinent to the

genesis of arrhythmias after Mustards
operation or the Senning procedure. It had
been suggested that the arrhythmias are
due to damage to purported specialised
internodal pathways29. As we have
explained in Chapter 2, there are no
insulated tracts to be found within the atrial
myocardium. Instead, the atrioventricular
impulse is conducted from the sinus node
through the thicker muscles of the right
atrial wall and septum, with the anisotropic
aggregation of the cardiomyocytes
favouring preferential conduction
(Figure 8.47). It is advantageous, therefore,
to preserve at least one of these routes. If
the terminal crest is to be divided to enlarge
the new pulmonary venous atrium, this is a
further reason to preserve the superior
border of the oval fossa. These procedures,
together with scrupulous avoidance of the
sinus node and its blood supply, mean that
arrhythmias can be reduced considerably,
if not totally avoided, after atrial redirection
procedures.
It is always important to avoid the
atrioventricular node. The landmarks to
this vital structure are the same as in the
normal heart. Providing all surgery is
performed outside the triangle of Koch,
injury to the node will be avoided. The
technique of cutting back the coronary
anatomically, by removing the endocardium from the inner surface

of the right atrium. It shows the non-uniform anisotropic
arrangement of the aggregated cardiomyocytes making up the
atrial walls. There is preferential conduction along the major axis of
muscle bundles such as the terminal crest, but no insulated tracts
within the atrial musculature. The site of the atrioventricular node,
at the apex of the triangle of Koch, is shown by the star.
sinus should be carefully considered. It is

possible to perform this procedure without
damaging the node and its zones of
transitional cells, but the incision will
undoubtedly cross one of the preferential
routes of conduction. For this reason, it is
safer to place the inferior suture line so as to
avoid the triangle of Koch completely.
It is also important to place the
interatrial bafes so as to minimise the risk
of subsequent venous obstruction, either in
the pulmonary or venous pathways.
Although it was suggested that venous
obstruction was less likely to complicate the
Senning procedure, those who became
skilled in the Mustard procedure were able
to achieve excellent results with a minimal
incidence of late venous obstruction30.
Almost certainly, therefore, it is the
surgical technique that determines the
likelihood of postoperative venous
obstruction.
The major complicating lesion in
transposition is the presence of a
ventricular septal defect, with or without
obstruction of the left ventricular outow
tract. Any other lesion can coexist if
anatomically possible. The anatomy of the
additional lesions is then as described for
the lesion found in isolation.
It follows, therefore, that ventricular
septal defects, when found in patients with
transposition, can be just as variable as

when found in isolation (Figure 8.48). The
majority of defects open between the outlet
components of the ventricular mass, but
have their own peculiar characteristics. In
such settings, typically with malalignment
of the outlet septum, they can extend to
become perimembranous when there is
brous continuity between the leaets of
the tricuspid and pulmonary valves
(Figure 8.49), or have a muscular
posteroinferior rim (Figure 8.50). The
distinguishing feature, as in tetralogy of
Fallot, is whether the posterocaudal limb of
the septomarginal trabeculation, or septal
band, fuses with the ventriculoinfundibular
fold. If there is fusion (Figure 8.50), the
resultant muscle bar will buttress the
conduction axis, and there will be
discontinuity between the leaets of the
tricuspid and pulmonary valves. If there is
no fusion of the muscular bars, the defect
will be perimembranous. The penetrating
atrioventricular bundle will thus be at risk
in this area of brous continuity
(Figure 8.49).
These defects have other features of
surgical signicance. The tension
apparatus of the tricuspid valve tends to
course over the defect, attaching itself to
the outlet septum or to the
ventriculoinfundibular fold. This makes
271
Doubly committed &

juxta-arterial
Aorta
Malalignment
Perimembranous
Muscular
Fig. 8.48 The cartoon shows how the ventricular septal defect in
hearts with transposition, as in the normal heart, can be
perimembranous, muscular, or doubly committed and juxtaarterial. The cartoon also emphasises the potential for
malalignment of the muscular outlet septum, a particular feature
of the ventricular septal defect in the setting of transposition.
Muscular
outlet septum
Aorta
Tricuspidpulm. continuity
Fig. 8.49 This view of a specimen with transposition, seen in
Tricuspid
valve
Sup.
Left
Right
Inf.
closure of the defect difcult without

producing damage to the tricuspid valve,
albeit that this is of less signicance when
the tricuspid valve is in the pulmonary
anatomical orientation from the right ventricle, shows the right

ventricular aspect of a perimembranous defect (star). The defect
opens into the subaortic outlet with malalignment of the outlet
septum, with overriding of the leaets of the pulmonary valve.
Note the brous continuity posteroinferiorly between the leaets
of the pulmonary (pulm.) and tricuspid valves. The conduction axis
is shown by the red dotted line.
circuit after an arterial switch. Of more

signicance is the divergence of the
margins of the parietal and anterior heart
walls. In hearts with concordant
ventriculoarterial connections, the anterior

margin of a ventricular septal defect is
usually well circumscribed. In the setting of
transposition, the muscular outlet septum
272
fold
Aorta
Malaligned muscular
outlet septum
Caudal limb
of SMT
Fig. 8.50 This specimen, seen in anatomical orientation from the

Sup.
Tricuspid valve
Right
Left
Inf.
right side, has a ventricular septal defect (star) opening into the
outlet of the right ventricle as in Figure 8.49, but with a muscular
posteroinferior rim produced by continuity between the caudal
limb of the septomarginal trabeculation (SMT) and the
ventriculoinfundibular fold. Note the malalignment of the outlet
septum. The muscular rim protects the conduction axis, shown by
the red dotted line.
Aorta
Pulmonary
-mitral continuity
SMT
Sup.
Fig. 8.51 In this heart with transposition, seen in anatomical
Tricuspid valve
Right
Left
Inf.
is frequently malaligned relative to the rest

of the muscular ventricular septum as it
inserts into the anterocephalad wall of the
right ventricular outow tract. The defect,
therefore, is more difcult to close at this
anterior margin (Figures 8.49, 8.50).
orientation from the right side, a perimembranous defect opens

into the inlet of the right ventricle in the absence of malalignment
of the muscular outlet septum. The conduction axis, shown by the
red dotted line, is deviated posteroinferiorly, but still relates
directly to the rim of the defect. SMT, septomarginal trabeculation.
Defects can open into the inlet of the

right ventricle, and again be
perimembranous (Figure 8.51) or muscular
(Figure 8.52). Some muscular defects open
centrally, while others open apically, often
being multiple. Defects opening into the
ventricular inlet, which are also

perimembranous, are the harbingers of
valve. This should always be suspected
when the right ventricle is hypoplastic.
Straddling and overriding of the tricuspid
273
Sup.
Left
Right
Inf.
Right atrium
Fig. 8.52 This heart, again with transposition, and photographed

Muscular ventricular septal defect
through the right atrioventricular junction, has a muscular defect

opening into the inlet of the right ventricle. In this setting, the
conduction axis, shown by the red dotted line, courses
anterosuperior relative to the defect.
Sup.
Oval fossa
Right
Left
Inf.
Fig. 8.53 In this heart with transposition, shown in anatomical
AV node displaced
posteroinferiorly
Straddling and overriding

tricuspid valve
valve markedly increases the risks of

surgery, not least because of the abnormal
disposition of the conduction tissues
(Figures 8.53, 8.54). Doubly committed
orientation, there is straddling and overriding of the tricuspid

valve. Because of the malalignment between the atrial and
ventricular septums, the atrioventricular (AV) node (red circle) is no
longer at the apex of the triangle of Koch (star), being deviated
posteroinferiorly, as shown by the superimposed course of the
conduction axis (red dotted line).
and juxta-arterial defects can also be found,

but are the rarest variant (Figure 8.55).
Subpulmonary obstruction in
transposition (Figure 8.56) can be
produced by any lesion which, in the

normal heart, would produce subaortic
obstruction. Valvar stenosis often
accompanies the subvalvar abnormalities.
274
Malaligned
ventricular
septum
Overriding right AV junction
Anomalous
AV node
Apex
Sup.
Regular AV node
(no contact)
Inf.
Base
Sup.
location of the conduction axis in hearts with malalignment between

the atrial and muscular ventricular septums, as found when there is
straddling and overriding of the tricuspid valve. AV, atrioventricular.
Aortic-pulm.
continuity
Aorta
Right
Fig. 8.54 The cartoon, shown in surgical orientation, illustrates the
Left
Inf.
SMT
Tricuspid
valve
Tricuspid-pulmonary continuity
More rare lesions, such as anomalous

insertion of the tension apparatus of the
atrioventricular valve, are probably beyond
surgical repair. Aneurysm of the
membranous septum, or similar brous
tissue tags (Figure 8.57) should be readily
amenable to removal, although a
subpulmonary brous shelf poses more
problems as it directly overlies the left
Fig. 8.55 In this heart with transposition, seen in anatomical

orientation from the front, the ventricular septal defect (star) is
doubly committed and juxta-arterial, with brous continuity
between the leaets of the aortic and pulmonary (pulm.) valves.
The defect also extends to become perimembranous. Note the
pulmonary-to-tricuspid brous continuity, which is reinforced with
a membranous ap. SMT, septomarginal trabeculation.
bundle branch. The lesion also typically

extends on to the pulmonary leaet of the
mitral valve, and can coexist with a
ventricular septal defect (Figure 8.58). The
difculties are compounded when the
brous obstruction is more extensive,
because it can form a subvalvar tunnel. The
other quadrants of the outow tract are also
vulnerable because of their proximity to the
left coronary artery, or because they are

formed by the pulmonary leaet of the
mitral valve. The safest area for resection is
beneath the remnant of the left margin of
the ventriculoinfundibular fold. When
subpulmonary obstruction coexists with a
ventricular septal defect, there is frequently
posterocaudal deviation of the outlet
septum, which is inserted in the left
Deviated outlet septum
275
Valvar
Tissue tags
Septal bulge
Abnormal
tension apparatus
Fibrous shelf
Fig. 8.56 The cartoon shows the lesions that can produce subpulmonary
obstruction in the setting of transposition. Exactly the same lesions will
produce subaortic obstruction in hearts with concordant ventriculoarterial
connections.
Sup.
Tissue tag
Pulmonary trunk
Ant.
Post.
Inf.
Mitral valve
Fig. 8.57 This view of the left ventricle from a specimen with
transposition, seen in anatomical orientation from the left, shows a
tissue tag from the septal leaet of the tricuspid valve herniating
through a perimembranous ventricular septal defect and
producing subpulmonary obstruction.
Left ventricle
ventricle (Figure 8.59). This usually means

that the aortic valve overrides the septum.
When the septal defect is substantial with
this combination, the scene is set for an
operative procedure such as the Rastelli or
Nikaidoh operations. When placing the
aortic outlet into the left ventricle during
these procedures, it is possible to resect the
outlet septum safely, which never harbours
conduction tissue. Such resection of the
outlet septum is also part of the REV

procedure. Should the defect be situated
other than between the ventricular outlets,
the chances of successfully transferring the
aortic infundibulum into the left ventricle
are considerably reduced.
Thus far, we have devoted attention
exclusively to the segmental anatomy of
transposition. There is further variation to
be found in terms of arterial relationships
and infundibular anatomy. These

variations do not alter the intracardiac
anatomy, and are of relatively minor
surgical signicance. For example, the
aorta is usually anterior and to the right in
complete transposition (Figure 8.60) but as
already illustrated, the aorta may be
anterior and to the left. This is the rule
when the atrial chambers are mirrorimaged (Figure 8.44), but left-sided and
276
Sup.
Ant.
Pulmonary trunk
Post.
Inf.
Left ventricle
Mitral
valve
Fig. 8.58 In this view of the left ventricular outow tract of a

specimen with transposition, seen in anatomical orientation, there
is obstruction due to an extensive brous shelf that extends onto
the leaet of the mitral valve (arrows).
VSD
Deviated
outlet
septum
Left ventricle
Sup.
Ant.
Post.
Inf.
Fig. 8.59 This specimen of transposition, seen from the left

Narrowed subpulm. outlet
transposed aortas can also be encountered

when there is usual atrial arrangement
(Figure 8.43). In even more rare cases, the
aorta can be posterior and to the right of the
ventricle in anatomical orientation, has posterocaudal deviation of

the outlet septum through a coexisting ventricular septal defect.
pulmonary trunk (Figure 8.61). These

different relationships do not alter the basic
anatomy. They do show why it is
inadvisable to use the term d-transposition
as a unifying terminology, as it makes little

sense to use this term for description of the
patient in whom the aorta is left-sided
when there is usual atrial arrangement.
277
Pulmonary
trunk
Sup.
Left
Right
Anterior
right-sided
aorta
Inf.
Right posterior aorta
Fig. 8.60 This heart is shown in anatomical orientation,

demonstrating the usual relationship of the aorta in the setting of
transposition, namely anterior and to the right of the pulmonary
trunk. Note the parallel arrangement of the arterial trunks as they
extend into the mediastinum.
Anterior
spiralling
pulm. trunk
Fig. 8.61 In this specimen of transposition, shown in anatomical

Sup.
Left
Right
Inf.
There are some clues to associated

lesions to be drawn from these unexpected
arterial relationships. When the aorta is
left-sided with the usual atrial
orientation from the front, the aorta is positioned posteriorly and

in a rightwards position relative to the pulmonary trunk, with
spiralling of the arterial trunks. This is the normal relationship of
the arterial trunks, but still with discordant ventriculoarterial
connections. The arrows show that the right ventricle supports the
aorta, while the left ventricle gives rise to the pulmonary (pulm.)
trunk.
arrangement, any coexisting ventricular

septal defect is frequently doubly
committed and juxta-arterial. This
arrangement is convenient for direct
connection of the aorta to the left ventricle.

When the aorta is posterior and the arterial
trunks are normally related, there is usually
a subpulmonary infundibulum, with the
278
Sup.
Left
Right
Pulmonary trunk
Inf.
Mitral
valve
Left ventricle
Fig. 8.62 This heart, photographed through the left ventricle in

anatomical orientation, shows a large subpulmonary infundibulum
(white double-headed arrow) separating the leaets of the
pulmonary and mitral valves.
leaets of the aortic valve in brous

continuity with the anterior leaet of the
mitral valve through the roof of a
perimembranous septal defect. This
unusual anatomy can create difculty both
at initial diagnosis and at subsequent
surgery. Variations in infundibular
morphology in themselves are unlikely to
give problems. The expected subaortic
muscular infundibulum in the right
ventricle is encountered most frequently
along with brous continuity between the
leaets of the pulmonary and mitral valves
in the left ventricle. Rarely, there may be a
complete muscular infundibulum in both
ventricles (Figure 8.62). Even more rarely,
as described earlier, there may be a
subpulmonary infundibulum with
and mitral valves, particularly when the
discordantly connected aorta is in a
posterior position.
The feature currently of greatest
surgical importance in patients with
transposition is the morphology of the
coronary arteries. This is because of the
universal acceptance of the arterial switch
procedure as the surgical treatment of
choice. This operation involves transecting
the ascending aorta and pulmonary trunk,
and reconnecting them to their

The signicant morphological feature is
that, thus far without reported exception,
the coronary arteries arise from the aortic
sinuses that are adjacent to the pulmonary
trunk (Figure 8.63). When performing the
arterial switch, therefore, the surgeon is
required to transfer the origins of the
coronary arteries across a relatively short
distance (Figure 8.64). This holds true
irrespective of the relationship of the aorta
to the pulmonary trunk.
The variable relationships between the
aorta and the pulmonary trunk, however,
produce problems in naming the aortic
sinuses and, hence, the origin of the
coronary arteries. Truly formidable
conventions are created if attempts are
made to catalogue each and every pattern31,
while use of simple alphabetical codes32 is
self-evidently procrustean. As suggested
by the group from Leiden33, it is best to
name the aortic sinuses as viewed from the
stance of the observer standing in the nonadjacent sinus of the aorta and looking
towards the pulmonary trunk
(Figure 8.65). Thus, one sinus is always to
the observers right-hand side. This is
designated as sinus no.1. The other sinus is
to the left-hand side, and is called sinus
no.2. The three major coronary arteries can
arise from either of these sinuses, giving

only eight potential patterns of sinusal
origin, including the arrangements in
which both arteries arise from the same
aortic sinus (Figure 8.66). Thus far, only
seven have been seen in the setting of
transposition34. Transfer of the arteries
during the switch procedure can be
compromised by an anomalous course of
the coronary arteries themselves in
relation to the vascular pedicle
(Figure 8.67), albeit that such variations
can be suitably accommodated by
appropriate surgical technique. An
intramural course of the origin of the
coronary artery across a valvar
commissure (Figure 8.66), or through the
aortic wall because of a high origin
(Figures 8.68, 8.69), creates greater
problems, but these can also be resolved
now with appropriate surgical technique.
The origin of the artery to the sinus node
can be very close to the origin of one
coronary artery from an aortic sinus, or the
sinus nodal artery can originate separately
from the sinus. Also of note is mismatch
between the commissures of the aortic and
pulmonary valves (Figure 8.70)35. Despite
all these anatomical variations, surgeons
now nd it possible to perform the arterial
switch procedure with remarkable success.
279
Aorta
Pulm.
trunk
Tricuspid
valve
Mitral
valve
Sup.
Right
Left
Inf.
anatomical orientation, the atrial myocardium and the arterial

trunks having been removed. The ventriculoarterial connections
are discordant. The dissection shows how the coronary arteries
(arrows) arise from the two aortic sinuses that are adjacent to the
pulmonary (Pulm.) trunk.
Aortic root
Left
Sup.
Fig. 8.63 In this heart, the short axis is viewed from above in
Inf.
Right
Fig. 8.64 The picture, taken in the operating room, shows the
Pulmonary root
Right coronary arterial button
CONGENITALLY CORRECTED
TRANSPOSITION
We describe the combination of discordant
connections as congenitally corrected
transposition (Figure 8.71). When dened
short distance required to transfer a button supporting one of the

coronary arteries from the old aortic to the old pulmonary root
during the arterial switch procedure.
in this fashion, as with transposition itself

(Figure 8.42), the entity can exist with the
atrial chambers in their usual arrangement,
or in mirror-imaged position
(Figures 8.728.74). Furthermore, like
transposition, it cannot exist when there is
isomerism of the atrial appendages,
although hearts with isomeric appendages

can have left-hand ventricular topology in
association with discordant
ventriculoarterial connections.
The complexities of congenitally
corrected transposition reect the fact that,
with the usual atrial arrangement, there is
280
Left-hand facing sinus

no. 2
Pulmonary trunk
Right-hand facing sinus
no. 1
Sup.
Ant.
Post.
Non-adjacent sinus

no. 2
Inf.
Fig. 8.65 The cartoon shows how the two aortic sinuses
that face the pulmonary trunk can always be described
accurately as being to the surgeons left-hand or right-hand
side when standing in the non-adjacent sinus of the aorta
and looking towards the pulmonary trunk, irrespective of
the relationships of the arterial trunks. Conventionally, the
sinus to the right-hand side is described as being no. 1, while
that to the left-hand side is designated as being no. 2.

no. 1
LCA
RCA
Fig. 8.66 In this heart from a patient with transposition, the aortic
Sup.
Left
Right
Non-adjacent sinus
Inf.
malalignment between the inlet part of the

muscular ventricular septum and the atrial
septum. These two septal structures are in
line at the crux but, when traced forwards,
they diverge markedly. This produces a
gap, into which is wedged the
subpulmonary outow tract from the
morphologically left ventricle
root has been opened and is viewed from the front. Both of the
coronary arteries arise from the left-hand facing sinus, or sinus no.
2. The main stem of the left coronary artery (LCA) was noted to take
an intramural course as it extended towards the left
atrioventricular and interventricular grooves. RCA, right coronary
artery.
(Figure 8.75). This abnormal feature

accounts for the most important surgical
aspect of congenitally corrected
transposition, namely the unusual
disposition of the atrioventricular
conduction tissues36. Because of the septal
malalignment, it is not possible for the
normal atrioventricular node, positioned at
the apex of the triangle of Koch, to

penetrate through the brous
atrioventricular junction and make contact
with the ventricular conduction tissues.
Instead, an anomalous atrioventricular
node, found in an anterolateral position
(Figure 8.76), as in the double-inlet left
ventricle (see Figure 8.14), gives rise to the
Right coronary
artery
Circumflex
artery
Solitary arterial stem

from sinus no. 1
Pulmonary
trunk
Aorta
Fig. 8.67 In this heart, photographed in anatomical orientation
Anterior
interventricular
artery

no. 2
281
LCA
from the front, the right and left coronary arteries arise from a
solitary vessel, with the right coronary and circumex arteries then
passing in front of the arterial pedicle.

no. 1
RCA
Sup.
Fig. 8.68 In this heart, as in the specimen shown in Figure 8.66,

Left
Right
Non-adjacent sinus
Inf.
penetrating atrioventricular bundle. The

bundle penetrates the insulating plane of
the atrioventricular junction lateral to the
area of brous continuity between the
leaets of the pulmonary and mitral valves.
A long non-branching bundle then runs
around the anterior quadrants of the
both coronary arteries arise within the left-hand facing sinus. In this
heart, the orice of the main stem of the left coronary artery (LCA)
is above the sinutubular junction. Having exited from the aortic
root, the artery courses between the arterial trunks in intramural
fashion (see Figure 8.69). RCA, right coronary artery.
subpulmonary outow tract, crossing the

characteristic anterior recess of the
morphologically left ventricle, before
descending onto the muscular ventricular
septum (Figure 8.77). Having branched,
the fan-like left branch is distributed in the
right-sided morphologically left ventricle,
while the cord-like right branch of the

bundle penetrates the septum to reach the
left-sided morphologically right
ventricle36. The discordantly connected
aorta arises from this morphologically right
ventricle, typically above a complete
muscular infundibulum, and usually in a
282
Sup.
Left
Right
Inf.
Pulmonary trunk
Aorta
Intramural
course
Fig. 8.69 The heart shown in Figure 8.68 is photographed from

Circumflex artery
the left side, showing how the main stem of the left coronary artery
is embedded within the wall of the aorta as it runs towards the
anterior surface of the heart, where it divides into its circumex and
anterior interventricular branches.
Aorta
Pulmonary
trunk
Fig. 8.70 In this heart with transposition, in which the atrial

myocardium and the arterial trunks have been removed, and being
photographed from the atrial aspect, there is mismatch between
the zones of apposition of the leaets of the aortic and pulmonary
valves (stars). Because of this, the distance for transfer of the right
coronary artery is increased (double-headed arrow).
left-sided position (Figure 8.78). When

congenitally corrected transposition is
found in the mirror-imaged variant,
however, it is the rule for the aorta to be
located in an anterior and right-sided
position (Figure 8.74). This is why it is
less than ideal to use d-transposition as

an alternative term for regular
transposition.
When congenitally corrected
transposition exists without any other
anomaly, the circulation of the blood is
normal. This situation is very much the

exception. Usually, one or more of three
associated lesions are found. These are a
ventricular septal defect, pulmonary
stenosis, or anomalies of the left-sided
atrioventricular valve37. It is these
Aorta
Aorta
Morph.
right atrium
Morph.
left atrium
Morph.
left atrium
Pulm.
trunk
283
Pulm.
trunk
Morph.
right atrium
Morph.
right ventricle
Fig. 8.71 The cartoon shows the
Morph.
right ventricle
Morph.
left ventricle
segmental combinations of
discordant atrioventricular and
that produce the lesion best
described as congenitally
corrected transposition. As
shown, the arrangement can be
found in usual and mirror-imaged
variants. Morph.,
morphologically; Pulm.,
pulmonary.
Morph.
left ventricle
Usual atrial arrangement
Mirror-imaged atrial arrangement
Aortic root
Right-sided
morph. LA
Morph. RCA
Sup.
Fig. 8.72 The computed tomographic angiogram, viewed from

Right-sided
morph. RV
Ant.
Post
Inf.
associated lesions that require surgical

treatment.
A ventricular septal defect is present in
up to three-quarters of patients. As in the
the right side, shows a right-sided morphologically left atrium

(morph. LA) that is connected to a right-sided morphologically right
ventricle (RV), which then supports the aorta. This is congenitally
corrected transposition in the setting of mirror-imaged
arrangement of the organs. RCA, right coronary artery.
patient with concordant atrioventricular

connections, it may be perimembranous,
muscular, or doubly committed and juxtaarterial (Figure 8.79). Usually it is of the
perimembranous type, and opens between

the ventricular inlets. Because of the
wedged position of the pulmonary valve,
the pulmonary trunk tends to override the
284
Sup.
Right
Right-sided
morph. LA
Left
Inf.
Left-sided
morph. RA
Right-sided
morph. RV
Left-sided
morph. LV
Fig. 8.73 The image shows the four-chamber cut through the
computed tomographic angiographic dataset shown in Figure 8.72.
The narrow neck of the right-sided atrial appendage (star) shows
that it has left morphology (morph. LA), while the other
appendage has pectinate muscles extending to the crux (white
arrow) conrming the left-sided location of the morphologically
right atrium (morph. RA). The nature of the apical trabeculations
conrm that the atrial chambers are connected to inappropriate
morphological ventricles (morph. LV, morph. RV).
Aorta
Pulmonary
trunk
Ant.
Right
Left
Post.
Fig. 8.74 The short axis cut through the dataset created from the
computed tomographic angiograms shown in Figures 8.72 and 8.73
shows the right-sided and anterior location of the aorta relative to
the pulmonary trunk. This shows that d-transposition can also be
congenitally corrected. Note the normal position of the coronary
arteries because of the right-handed ventricular topology that is a
feature of this segmental combination.
285
Sup.
Plane of atrial septum
Left
Right
Right
atrium
Inf.
Fig. 8.75 This specimen with congenitally corrected

transposition, viewed in anatomical orientation from the
morphologically (Morph.) left ventricle, shows the malalignment
between the atrial (yellow double-headed arrow) and ventricular
(red double-headed arrow) septal structures, with the
subpulmonary outow tract wedged into this gap (white doubleheaded arrow). The site of the atrioventricular conduction axis is
shown by the superimposed green loop. (Reproduced by kind
permission of Prof. Anton Becker, University of Amsterdam.)
Morph. left
ventricle
Plane of ventricular septum
Anterolateral node
Left
Mitral valve
Sup.
Inf.
Right
Aorta
Pulm.
trunk
Fig. 8.76 The cartoon shows the view of the morphologically
Pulmonary valve
Connecting node not

in triangle of Koch
defect (Figure 8.80). When seen from the

right atrium, it is shielded by the superior
end of the zone of apposition between the
leaets of the right-sided morphologically
mitral valve in congenitally corrected transposition as seen by the

surgeon working through a right atriotomy. The locations of the
atrioventricular node and the conduction axis have been marked in
red. There is a perimembranous ventricular septal defect. Pulm.,
pulmonary.
mitral valve (Figure 8.81). The abnormal

position of the atrioventricular node and
the non-branching atrioventricular
bundle must be borne in mind when
closing the defect. The atrioventricular

bundle arises from the anomalous
anterolateral node, penetrates the area of
pulmonary-mitral valvar continuity, and
286
Left
Sup.
Inf.
Right
Morph. left ventricle
Aorta
Fig. 8.77 In this cartoon, we show the view of a perimembranous

ventricular septal defect in congenitally corrected transposition, as
would be seen by the surgeon working through a generous incision in
the right-sided morphologically (Morph.) left ventricle. The course of
the conduction axis has been marked in green. Pulm., pulmonary.
Pulm.
trunk
Pulmonary valve
Sup.
Pulmonary trunk
Left
Right
Inf.
Aorta
Morphologically
right appendage
Morphologically
right ventricle
Fig. 8.78 This specimen is photographed in anatomical

orientation from the front, showing the usual left-sided location of
the aorta relative to the pulmonary trunk in patients with
congenitally corrected transposition.
then runs around the subpulmonary

outow tract to descend on the
anterosuperior margin of the defect
(Figure 8.77).
The safest way of closing the defect
without damaging the conduction system is
to place sutures from the left-sided
morphologically right ventricular aspect
(Figure 8.82)38. Although the defect opens
mostly between the ventricular inlets, it can

extend to become doubly committed and
juxta-arterial. It is then roofed by the
conjoined leaets of the aortic and
pulmonary valves (Figure 8.83). When it is
perimembranous and juxta-arterial, the
conduction tissue remains in an anterior
position. In some patients, however,
there can be a muscular inferior rim
between the leaets of the pulmonary

and mitral valves. It is still likely that the
conduction tissue remains in an
anterosuperior position relative to the
defect. A comparable case has been seen
with both a perimembranous and a
muscular outlet defect. The conduction
axis descended along the muscle bar
between them.
287
Valvar
continuity
Doubly committed
and juxta-arterial
Perimembranous
Pulmonary
valve
Tricuspid
valve
Pulm.
trunk
Mitral
valve
Morphologically
left ventricle
Sup.
Muscular
Ventricular
septal
defect
Fig. 8.79 The cartoon shows that, in congenitally corrected

transposition as in the otherwise normal heart, the ventricular
septal defect can be perimembranous, muscular, or doubly
committed and juxta-arterial. In congenitally corrected
transposition, however, the conduction axis, shown in yellow,
takes a grossly abnormal course relative to the defects. Pulm.,
pulmonary.
Right
Left
Inf.
Fig. 8.80 This view of a specimen with congenitally corrected

transposition, shown in anatomical orientation from the front, illustrates the
relationships of a perimembranous ventricular septal defect opening into
the inlet of the morphologically left ventricle.
Left
Right-sided
mitral valve
Inf.
Sup.
Right
Coronary sinus
Anomalous
anterior node
and bundle
Regular
node in triangle
of Koch
(no contact)
Fig. 8.81 This view of a perimembranous inlet ventricular septal

defect in congenitally corrected transposition taken in the
operating room, as seen through the right-sided morphologically
mitral valve, shows how the defect and the subpulmonary outow
tract are shielded behind the anteroseptal commissure of the valve.
The cross-hatched areas show the locations of the connecting and
non-connecting atrioventricular nodes. (Reproduced by kind
permission of Dr. Jan Quaegebeur, Columbia University, New
York.)
288
Left
Ventricular
septal defect
Inf.
Sup.
Right
Fig. 8.82 This further view of the patient shown in Figure 8.81
Needle on
left side
of septum
Coronary
sinus
Pulmonary
trunk
Sup.
illustrates how the axis of conduction tissue (cross-hatched area)

can be avoided by placing sutures through the defect on its
morphologically right ventricular aspect, which is left-sided when,
as in this patient, there was the usual atrial arrangement.
(Reproduced by kind permission of Dr. Jan Quaegebeur, Columbia
University, New York.)
Pulmonary-aortic
continuity
Left
Right
Inf.
Mitral
valve
Fig. 8.83 In this specimen, viewed in anatomical orientation, the

Morphologically
left ventricle
Pulmonary-tricuspid
continuity
As with transposition, any of the lesions

that produces obstruction of the left
ventricular outow tract will produce
pulmonary stenosis when the transposition
is congenitally corrected (Figure 8.84).
perimembranous ventricular septal defect (star) extends to become

doubly committed and juxta-arterial, being roofed by a brous
continuity between the leaets of the aortic and pulmonary valves.
Valvar stenosis in isolation is rare, but

frequently coexists with subvalvar
obstructive lesions. Particularly signicant
in this respect are brous tissue tags
(Figure 8.85). Fibrous diaphragmatic
lesions (Figure 8.86), or muscular

obstruction, are also encountered39. The
overwhelming consideration in all of
these types of stenosis is the
presence of the non-branching bundle
289
Valvar stenosis
Pulmonary
trunk
Tissue tags
Fig. 8.84 The cartoon shows the lesions that can produce
subpulmonary obstruction in the setting of congenitally corrected
transposition. Note the anterior position of the conduction axis,
shown in yellow. As with transposition, the same lesions produce
subaortic obstruction in the setting of concordant
ventriculoarterial connections.
Fibrous shelf
Sup.
Left
Right
Pulmonary
trunk
Inf.
Mitral
valve
Morphologically
left ventricle
running around the anterior quadrants

of the outow tract. Apart from tissue
tags, this relationship makes it very
difcult to resect these various lesions.
Placement of a conduit may be the safest
means of avoiding postoperative heart
block.
Fig. 8.85 In this specimen, viewed in anatomical orientation

through the morphologically left ventricle, a brous tissue tag
derived from the septal leaet of the left-sided morphologically
tricuspid valve (star) herniates into the subpulmonary outow
tract.
Ebsteins malformation is the lesion that

most frequently aficts the left-sided
morphologically tricuspid valve, involving
downwards displacement of the septal and
mural leaets (Figure 8.87). Only rarely is
the inlet part of the ventricle dilated and
thinned, as occurs so frequently in
Ebsteins malformation with concordant

atrioventricular connections. Should
replacement of the valve become
necessary, the location of the conduction
tissues in the right-sided atrioventricular
junction takes them out of the area of
danger.
290
Sup.
Site of anterior
node
Left
Right
Inf.
Mitral
valve
Fig. 8.86 In this specimen, seen in anatomical orientation

Subpulmonary
fibrous ring
Perimembranous
ventricular septal defect
through the morphologically left ventricle, a brous shelf produces

subpulmonary obstruction in the presence of a perimembranous
ventricular septal defect. The site of the axis of atrioventricular
conduction tissue has been superimposed in the cross-hatched
areas.
Atrialised
ventricle
Morphologically left atrium
Morphologically
right ventricle
Fig. 8.87 This specimen with congenitally corrected
Sup.
Left-sided
morphologically
tricuspid
valve
Post.
Ant.
Inf.
The other anomaly that affects the left

valve, and that can also affect the right
valve40, is straddling of its tension
apparatus and overriding of the valvar
orice (Figure 8.88). These anomalies do
not alter the origin of the conduction
transposition, seen in anatomical orientation from the left side,

shows Ebsteins malformation of the left-sided morphologically
tricuspid valve. There is no thinning of the musculature of the inlet
component of the morphologically right ventricle. The white
dotted line shows the location of the atrioventricular junction.
tissues from an anterolateral node, but will

increase the risks of surgery markedly.
Indeed, in the presence of straddling
atrioventricular valves, some may opt for
functionally univentricular rather than
biventricular surgical correction. Those
who use biventricular correction have

observed disappointing results
subsequent to simple correction of the
associated lesions, because these
manoeuvres leave the morphologically
right ventricle supporting the
291
Sup.
Post.
Ant.
Left atrium
Inf.
Fig. 8.88 In this heart with congenitally corrected transposition,

Hypoplastic
morphologically
right ventricle
Straddling and overriding

left-sided morphologically
tricuspid valve
systemic circulation. Because of this,

the trend now is to correct the defects
using the so-called double switch
procedure, combining atrial redirection
with either an arterial switch41 or use of an
interventricular tunnel to the aorta,
placing a conduit from the
morphologically right ventricle to the
pulmonary atrium42. This also makes it
possible to make corrections in
patients with coexisting pulmonary
atresia.
Some patients with discordant
atrioventricular connections will have
ventriculoarterial connections other than
discordant ones. We will discuss the
options for a double outlet from the
morphologically right ventricle in the next
section, but our emphasis there will be on
patients with concordant atrioventricular
connections. The variability to be
described can also be found when the
discordant, but then the atrioventricular
conduction axis will arise from an
anterolateral atrioventricular node, as
described for congenitally corrected
transposition. In patients with discordant
atrioventricular connections and a doubleoutlet right ventricle, nonetheless, it is also
viewed in anatomical orientation from the left-sided

morphologically right ventricle, there is straddling and overriding
of the left-sided morphologically tricuspid valve. Note the
hypoplastic nature of the morphologically right ventricle.
possible to nd a sling of conduction tissue

running along the crest of the muscular
ventricular septum and joining anterior and
regular atrioventricular nodes36. This
possibility should be borne in mind for all
patients having discordant atrioventricular
connections and a double outlet from the
morphologically right ventricle.
Some patients also have a single outlet in
association with discordant atrioventricular
connections, most frequently because of
pulmonary atresia (Figure 8.89). In this
situation, there is typically a
perimembranous ventricular septal defect.
If the defect is large, then, as we have
discussed, it is possible to achieve
biventricular repair by channelling the
morphologically left ventricle to the aorta,
placing a conduit from the morphologically
right ventricle to the pulmonary arteries,
and performing an atrial redirection
procedure42. The defect, however, is not
always large enough to permit channelling
of the morphologically left ventricle to the
aorta (Figure 8.90). If restrictive, the
decision must be made as to whether the
defect can be enlarged, or whether the
better option is to create the Fontan
circulation. The anterocephalad location of
the atrioventricular conduction axis in the
setting can make enlargement of the defect

a daunting procedure (Figure 8.90).
Exceedingly rarely, it is possible to nd
pulmonary atresia with an intact
ventricular septum and an imperforate
right-sided atrioventricular valve. When
seen from the right atrium, the
arrangement can be indistinguishable from
pulmonary atresia with an intact
ventricular septum (compare Figure 8.91
with Figure 8.31). The signicant
difference is that, unlike regular pulmonary
atresia with an intact ventricular septum, it
is the morphologically right ventricle that
must drive the systemic circulation when
the atrioventricular connections are
discordant (Figure 8.92). Aortic atresia, or
common arterial trunk, can also rarely be
found with discordant atrioventricular
connections. A more important
combination is that found with
concordant ventriculoarterial connections.
We will discuss this arrangement in
greater detail below. The signicant
surgical feature is that the combination
produces the ideal situation for
employment of an atrial redirection
procedure, because this restores the
morphologically left ventricle to pumping
the systemic circulation.
292
Left-sided anterior aorta
Sup.
Ant.
Post.
Inf.
Atretic pulmonary trunk
Fig. 8.89 The image shows the base of the heart. The atretic
Morph. right atrial appendage
Perimembranous VSD
Sup.
Post.
Ant.
pulmonary trunk is posterior and right-sided relative to the aorta.

The pulmonary arteries were fed through a persistently patent
arterial duct. Morph., morphologically.
Aorta
Inf.
Tricuspid valve
Left-sided morph.
right ventricle
DOUBLE-OUTLET VENTRICLE
It is a well-known fact that spectrums of
malformation exist in the settings of both
tetralogy of Fallot and transposition when
Fig. 8.90 The image shows the left-sided ventricle from the heart
illustrated in Figure 8.89. The atrioventricular connections are
discordant, and the aorta is supported by the left-sided
morphologically (morph.) right ventricle. The perimembranous
ventricular septal defect (VSD) is restrictive. The dotted red line
shows the course of the atrioventricular conduction axis, which is
carried on the right side of the ventricular septum. Its
anterocephalad location relative to the defect would make
enlargement difcult, if not impossible, to achieve without
producing atrioventricular block if the decision were made to
attempt biventricular repair by channelling the morphologically
left ventricle to the aorta, and then performing an atrial redirection
procedure.
there is overriding of the aortic and

pulmonary roots, respectively. It remains
controversial as how best to describe the
ends of these spectrums when the greater
part of the overriding arterial root arises
from the ventricle that is also supporting

the other arterial root. Our approach to this
dilemma is to argue that, when the greater
part of both arterial roots arises from the
same ventricle, then the ventriculoarterial
293
Imperforate mitral valve
SCV
Oval fossa
Fig. 8.91 The morphologically right atrium is photographed from
Sup.
Right
Left
Inf.
Coronary sinus
above and behind to reveal the presence of an imperforate

atrioventricular valve. When viewed in isolation, it is not possible to
distinguish the morphological nature of the valve. As shown in
Figure 8.92, however, the atrioventricular connections were
discordant, so the valvar membrane is morphologically mitral. SCV,
Sub-aortic infundibulum
Left-sided morph.
right ventricle
Tricuspid valve
Fig. 8.92 The image shows the left-sided ventricle in the heart
shown in Figure 8.91. The ventricle is coarsely trabeculated, and
receives the morphologically (morph.) tricuspid valve. The heart
had pulmonary atresia with an intact ventricular septum, but in the
setting of discordant atrioventricular and ventriculoarterial
connections.
Sup.
Post.
Ant.
Inf.
connection is that of a double outlet. On

this basis, we dene a double-outlet
ventricle whenever more than half of the
circumference of both arterial valves takes
its origin from the same ventricle43. In
many patients, this problem does not arise,
as both arterial trunks arise unequivocally

from the one ventricle. An additional
problem arises in this situation, because it
was initially considered that, to justify the
diagnosis of a double-outlet right ventricle,
not only did both arterial trunks need to be
supported exclusively from the right

ventricle, but also that each arterial valve
needed to have a completely muscular
infundibulum, or conus. This approach,
however, is inconsistent with the
morphological method, which states that
one variable feature, in this instance the

ventriculoarterial connection, should not
be dened on the basis of another feature
that is itself variable, namely the
infundibular morphology. It is now
accepted, therefore, that a double-outlet
right ventricle can exist in the absence of
bilateral infundibulums44. As we will show,
many hearts do indeed exist when the
arterial trunks arise from the right
ventricle, but with brous continuity
between the leaets of an arterial and an
atrioventricular valve. Furthermore, even
when there is overriding of an arterial root,
there is usually little question as to the
anatomical assignment of the abnormal
valve, because rarely does it appear to be
equally committed to both chambers. In
situations still creating difculty, the
decision regarding commitment of the
overriding root should be made according
to the connection of the leaets as seen in
short axis, rather than on the basis of the
alignment of the long axis of the ventricular
septum (Figure 8.93). Making designations
on the basis of long-axis views can be
misleading because of the motion of the
heart during the cardiac cycle. This does
not alter the arbitration as based on the
short axis. The 50% rule is simply a

pragmatic convention for distinguishing
between double-outlet and one-to-one
ventriculoarterial connections. When
based on such an approach, the topic of the
double-outlet ventricle becomes
straightforward, if not even simple.
The double-outlet ventriculoarterial
connection, dened as discussed previously,
accounts only for an anatomical arrangement
of the ventriculoarterial junctions. Hearts
with this feature, therefore, can exist with
such a wide variety of congurations that the
possible combinations seem almost limitless.
The cases to be illustrated in this section are
those with both arteries arising from the
right ventricle in the setting of concordant
atrioventricular connections. The principles
to be established are equally applicable in the
setting of the other atrioventricular
connections, remembering that when the
atrioventricular connections are discordant,
or there is left-hand topology in the setting of
isomerism, there is likely to be an
anomalously located atrioventricular node,
or even dual atrioventricular nodes. Almost
always, there is a coexisting interventricular
communication in the presence of the double
outlet45. It is thus the anatomical relationship
between the great arteries and the defect in

the ventricular septum that is important to
the surgeon. Naming the hole between the
ventricles creates an additional difculty. In
patients having a ventricular septal defect in
the setting of one-to-one ventriculoarterial
connections, the ventricular septal defect is
typically dened as the place of space limited
caudally by the crest of the muscular
ventricular septum, and cranially by the
undersurface of the muscular outlet septum
(Figure 8.94, left-hand panel). When both
arterial trunks are supported within the right
ventricle, the muscular outlet septum is
exclusively a right ventricular structure
(Figure 8.94, right-hand panel). The
analogous plane of space to the regular
ventricular septal defect would be the locus
over which a patch is placed to tunnel one or
the other arterial trunk into the left ventricle,
this being the therapeutic option usually
taken by the surgeon to restore biventricular
circulations. This plane, however, is not the
hole that is commonly dened as the
ventricular septal defect in this setting.
Instead, it is the exit from the left ventricle
that is typically considered to represent the
ventricular septal defect. Therefore, to avoid
confusion, we prefer to name the hole as the
True override
is assessed relative
to chord of circle
Artery
Short axis section
B
o S
in rien epta
sh ta l
or tio
ta n
xis
294
Fig. 8.93 The cartoon shows how overriding
True override
is independent of
septal axis
arterial valves are assigned to one or the other

ventricle on the basis of their connection as
judged in the short axis (left-hand panel), in
which it is possible to assess the tangent drawn
by the ventricular septum (XY) relative to the
short axis of the overriding arterial trunk,
rather than the long axis of the ventricular
septum (AB in the right-hand panel), which
can change depending on the motion of the
heart.
Ao
Ventricular
septal
defect
PT
Muscular
outlet septum
RV
Ao
PT
Interventricular
communication
LV
LV
RV
Crest of muscular
ventricular septum
Patch for biventricular connection
One-to-one
Double-outlet right ventricle
295
Fig. 8.94 The cartoon illustrates the

signicant difference between the plane of
space identied as the ventricular septal
defect when there are concordant or
discordant ventriculoarterial connections
(one-to-one connections) as opposed to a
double outlet from the right ventricle. The
left-hand panel illustrates the situation with
discordant ventriculoarterial connections, but
the concept is equally valid when the
connections are concordant. As shown in the
right-hand panel, the hole usually identied as
the ventricular septal defect is better
considered to be the interventricular
communication. Ao, aorta; PT, pulmonary
trunk; LV and RV, left and right ventricles.
Aorta
Pulm.
trunk
Morphologically
right ventricle
Sup.
Fig. 8.95 This specimen, shown in anatomical orientation from
Left
Right
the front, has a double outlet from the right ventricle with the
interventricular communication in subaortic position. The great
arterial trunks spiral in normal relationships as they leave the base
of the heart.
Inf.
interventricular communication.
Recognition of this fact also helps the
surgeon to determine whether a given
patient has a double-outlet ventriculoarterial
connection. Should it be considered
necessary to place a patch so as to tunnel the
communication to an arterial root, then the
patient will have had a double outlet. In

contrast, if the surgeon considers that it was
possible simply to close the septal defect,
then the patient will have had concordant or
discordant ventriculoarterial connections.
The relationship of the arterial trunks is
another variable in patients with a double
outlet from the right ventricle. In many, the

aorta and the pulmonary trunk are related
more or less normally. The aortic valve,
therefore, is posterior and to the right of
the pulmonary valve, and the pulmonary
trunk spirals around the aorta as it extends
towards its bifurcation (Figure 8.95). In the
296
Pulmonary
trunk
Aorta
Sup.
Left
Right
Inf.
Ao
PT
Fig. 8.96 In this specimen, seen in anatomical orientation from

the front and with a double outlet from the right ventricle, the
interventricular communication was in a subpulmonary position.
The arterial trunks rise in parallel fashion (black arrows) as they exit
from the base of the heart (compare with Figure 8.95).
PT
Ao
PT
Ao
Fig. 8.97 The cartoon, drawn in anatomical orientation, shows
Spiralling great
arteries
(normally related)
Great arteries
parallel
-aorta to right
remainder, the arterial trunks arise from the

base of the heart in parallel fashion, as
anticipated when the ventriculoarterial
connections are discordant. For a good
proportion of these, the arterial valves are
side-by-side (Figure 8.96); otherwise, the
aortic valve is anterior. In a few cases, the
aorta is posteriorly located because the
pulmonary trunk is enlarged. Almost
always, the aorta is to the right but, in a small
number of cases, the aorta can be left-sided
relative to the pulmonary trunk
(Figure 8.97). Although the interventricular
Great arteries
parallel
-aorta to left
the three typical orientations of the arterial trunks to be found in

hearts with a double-outlet right ventricle. The arrangements with
spiralling and parallel arterial trunks with the aorta to the right
have been shown in Figures 8.95 and 8.96. The rarest variant,
shown in the right-hand panel, is for the arterial trunks to rise in
parallel fashion, but with the aorta in the left-sided position. Ao,
aorta; PT, pulmonary trunk.
communication is most usually subaortic

when the arterial trunks are spiralling, and
subpulmonary when they extend into the
mediastinum in parallel fashion, there is
no direct correlation between these features.
It is always necessary, therefore, to
determine precisely the location of the
interventricular communication relative
to the arterial roots, and to establish
whether it can be channelled safely to one
or the other arterial root in an unobstructed
fashion so as to achieve a biventricular
surgical repair.
In the light of these discussions, it is

evident that the anatomy of the
interventricular communication needs to
be considered in two ways. The rst feature
of signicance is its proximity to the great
arteries45. The second feature concerns its
own intrinsic morphology. When
combining these two approaches, it must be
remembered that, when there is a double
outlet from the right ventricle, the
muscular ventricular septum itself
separates only the inlet and apical
trabecular components of the ventricles. As
Sup.
Pulmonary trunk
Left
Right
Aorta
297
Inf.
Muscular
outlet
septum
Caudal limb of SMT
we have emphasised, the outlet septum, or

its brous remnant, is found between the
two outlets from the right ventricle, and
does not form the roof of the
interventricular communication.
Except when non-committed, the
interventricular communication is cradled
between the limbs of the septomarginal
trabeculation, or septal band. Its other
borders vary depending on several features.
The rst is whether the caudal limb of the
septomarginal trabeculation fuses with the
ventriculoinfundibular fold. If it does,
there is a muscular inferior rim to the
defect. This protects the conduction tissue
axis as in tetralogy of Fallot or transposition
(Figure 8.98). If it does not, there is
continuity between the leaets of the
atrioventricular valves, and the defect is
perimembranous (Figure 8.99). The
second feature is the extent of the
ventriculoinfundibular fold. It is the
presence of well-formed folds bilaterally
that produces bilateral muscular
infundibulums, or the bilateral conuses that
were initially considered to be part-andparcel of the denition of the double-outlet
right ventricle. The signicant surgical
aspect of this feature is that the
infundibulums move the arterial valves
Fig. 8.98 In this specimen, shown in anatomical orientation from

the front, the interventricular communication (star) is cradled
within the limbs of the septomarginal trabeculation (SMT). Note
that the posterocaudal limb of the trabeculation fuses with the
ventriculoinfundibular fold. The muscular buttress thus formed
protects the atrioventricular conduction axis. There is brous
continuity between the leaets of the aortic and mitral valves in
the roof of the interventricular communication, so that it is directly
adjacent to the aortic root.
some distance away from the margins of the

interventricular communication
(Figure 8.100). Even in the setting of
bilateral infundibulums, the
interventricular communication can still be
perimembranous, as there can be
continuity between the leaets of the mitral
and tricuspid valves (Figures 8.100, 8.101).
When the ventriculoinfundibular fold is
attenuated, the leaets of one of the arterial
valves are able to achieve brous continuity
with an atrioventricular valve
(Figure 8.99).
The nal feature is the relationship of
the muscular outlet septum to the other
structures. When attached to the anterior
limb of the septomarginal trabeculation, the
defect is subaortic (Figures 8.988.101).
When, in contrast, the outlet septum is
attached to the ventriculoinfundibular fold,
or to the posterior limb of the
septomarginal trabeculation, the defect is
placed beneath the left-sided great artery,
which is almost always the pulmonary
trunk (Figure 8.102). When the outlet
septum is absent, the defect is doubly
committed and juxta-arterial
(Figure 8.103).
From this potential for variation, two
groups of patients can be collected from
those having a double outlet from the right

ventricle. The most frequent conguration
is when the aortic valve is posterior and to
the right of the pulmonary trunk
(Figure 8.95), usually with the
interventricular communication in a
subaortic position (Figures 8.988.102).
This arterial relationship can also be
found with a doubly committed and juxtaarterial defect (Figure 8.103). The
second group comprises hearts in which
the aorta ascends parallel to the pulmonary
trunk (Figure 8.96), usually with its
valve in either an anterior or side-by-side
position, and to the right relative to the
pulmonary valve, and with the septal
defect in a perimembranous or muscular
but subpulmonary position
(Figure 8.102). This is the TaussigBing
variant46.
The surgeon should be particularly
wary when the interventricular
communication is non-committed47.
Almost always, when it is subaortic,
subpulmonary, or doubly committed, it
is possible to place a patch within the
right ventricle so as to channel the
interventricular communication to one or
the other outow tract, thus achieving
biventricular circulations. This may not
298
Sup.
Pulmonary
trunk
Left
Right
Inf.
Aorta
Fig. 8.99 In this specimen, shown in anatomical orientation, the

Muscular
outlet
septum
Caudal limb of SMT
Muscular outlet septum
outlet septum is attached to the anterocephalad limb of the

septomarginal trabeculation (SMT) so that the defect (star) is
subaortic. There is extensive brous continuity between the leaets
of the mitral and tricuspid valves, making the defect
perimembranous, but there is also extensive continuity between
the leaets of the aortic and tricuspid valves (black dotted line),
even though the aortic root is supported exclusively within the
right ventricle. The specimen shows that hearts can have a double
outlet from the right ventricle without there being bilateral
infundibulums.
Sup.
Left
Right
Pulmonary
valve
Inf.
Aortic
valve
Morphologically
right ventricle
Tricuspid-mitral continuity
be possible if the defect is anatomically

non-committed, or if the interventricular
communication is adjacent to an outow
tract, but the potentially corrective
Fig. 8.100 In this specimen with a double-outlet right ventricle,

seen in anatomical orientation, there is an extensive subaortic
infundibulum, along with a long subpulmonary infundibulum
(double-headed arrows), but the defect is perimembranous, with
brous continuity between the leaets of the tricuspid and mitral
valves. The interventricular communication (star) is in the limbs of
the septomarginal trabeculation, and is adjacent to the subaortic
outow tract, but relatively distant from the aortic valve because of
the length of the subaortic infundibulum.
pathways are blocked by structures such as

valvar tension apparatus (Figure 8.104).
Anatomical non-commitment is most
usually found when the interventricular
communication is between the ventricular

inlets (Figure 8.104), or is the ventricular
component of an atrioventricular septal
defect in patients with a common
299
Pulmonary
trunk
Aorta
Fig. 8.101 In this specimen, seen in anatomical orientation, there
Sup.
Left
Right
Tricuspid-mitral
fibrous continuity
Inf.
is extensive brous continuity between the leaets of the tricuspid

and mitral valves, making the interventricular communication
perimembranous despite the presence of bilateral infundibulums
(black dotted line). Note that the subpulmonary infundibulum
(black brace) is narrowed by a squeeze between the outlet septum
and hypertrophied septoparietal trabeculations. Thus, the heart
has the phenotypical features of tetralogy of Fallot in addition to a
double-outlet connection and bilateral infundibulums. The star
shows the interventricular communication.
Sup.
Left
Right
Inf.
Pulmonary
trunk
Aorta
Tricuspid-mitral continuity

The surgeon may be able to overcome
these impediments and still achieve
biventricular repair48. In this situation,
however, there may be arguments as to
interventricular communication (star) is positioned in a

subpulmonary location. This is the so-called TaussigBing
malformation. Note the attachment of the muscular outlet septum
to the midpoint of the ventriculoinfundibular fold, along with
bilateral infundibulums (double-headed arrows). There is also
tricuspidmitral brous continuity, so the interventricular
communication is perimembranous.
whether the defect is truly non-committed

(Figure 8.106). There is no question but
that the defect is non-committed both
anatomically and surgically when it is
encased within the muscular septum and
opens into the inlet of the right ventricle

(Figure 8.107).
It is also important for the surgeon to
identify any other abnormalities in the
extremely heterogeneous group of
300
Aortic-pulmonary continuity
Pulmonary
valve
Aortic
valve
SMT
Sup.
Left
Right
Inf.
Aorta
interventricular communication (star) is doubly committed and

juxta-arterial due to the failure of formation of the subpulmonary
muscular infundibulum. There is brous continuity between the
leaets of the aortic and pulmonary valves, and brous continuity
between the aortic and tricuspid valves (red dotted line). SMT,
septomarginal trabeculation.
Outlet
septum
Pulm.
trunk
Sup.

Left
Right
Perimembranous VSD
Inf.
malformations linked together because of

a double-outlet ventriculoarterial
connection. By far the most common set
of anomalies relates to obstruction of the
arterial outlets, such as infundibular
interventricular communication opens between the ventricular

inlets, and is shielded by the septal leaet of the tricuspid valve.
This feature, coupled with the attachment of tendinous cords to
the muscular outlet septum (arrows), makes the defect noncommitted. VSD, ventricular septal defect.
stenosis, valvar stenosis, coarctation, or

interruption of the aortic arch. The
presence of subpulmonary infundibular
stenosis is a particularly frequent nding
when the septal defect is subaortic, often
producing the morphology of tetralogy of

Fallot, even in the setting of bilateral
infundibulums (Figure 8.101).
Coarctation and interrupted arch,
together with straddling of the mitral
301
Pulmonary
trunk
Aorta
Outlet
septum
Sup.
Left
Right
Atrioventricular
septal defect
Inf.
Fig. 8.105 In this specimen with a double-outlet right ventricle

and bilateral infundibulums (double-headed arrows), shown in
anatomical orientation, there is a common atrioventricular
junction guarded by a common atrioventricular valve. It is
questionable whether the ventricular component of the
atrioventricular septal defect could be channelled to the
subpulmonary root. Anatomically, the defect is non-committed.
Sup.
Pulmonary
trunk
Left
Right
Aorta
Inf.

(RV) and bilateral infundibulums (double-headed arrows), seen in
anatomical orientation, the interventricular communication is
again between the ventricular inlets (compare with Figure 8.105).
In this specimen, however, the inlet perimembranous defect could
almost certainly be committed surgically to the subpulmonary
outlet. In this instance, therefore, the defect might well be
described as being subpulmonary rather than non-committed.
Perimembranous defect
opening to inlet of RV
valve, are frequent accompaniments of the

TaussigBing variant46. Common
atrioventricular valves also occur in a
signicant number of cases. Unusual
coronary arterial anatomy, including the

intramural arrangement, is found most
frequently when the aorta is in an
anterior or side-by-side position. In this
situation, special consideration is needed

when performing either a right
ventriculotomy or an arterial switch
procedure.
302
Aorta
Pulmonary trunk

and bilateral infundibulums (double-headed arrows), shown in
anatomical orientation, the interventricular communication is
within the muscular septum and opens into the right ventricular
inlet. This defect is unequivocally non-committed.
Muscular inlet defect
Ventriculoinfundibular foldsafe for conduction tissue but

resection goes outside heart
Outlet septumsafe
Anterocephalad
limb-beware septal
perforator
Conduction axis
Fig. 8.108 The cartoon, illustrating a double-outlet right ventricle

Major danger with
arterial to tricuspid
continuity
Posterocaudal limbbeware
conduction axis
Surgical and anatomical considerations

for the potential corrective procedures
relate to the particular combination of
defects. It is possible, nonetheless, to
establish some basic rules (Figure 8.108)49.
The outlet septum, almost always present,
serves as a guide to the arterial valves and
with a subaortic interventricular communication, shows the major

components of the outow tracts, and illustrates the salient surgical
features relating to each individual muscular component. The
conduction axis is shown in red, and the rst septal perforating
artery as the red dotted line. The ventriculoinfundibular fold is
shown in green, the outlet septum in blue, and the septomarginal
trabeculation in yellow.
their relationships to each other, and to the

interventricular communication. As
described previously, in cases with
subaortic defects the outlet septum usually
inserts into the anterocephalad limb of the
septomarginal trabeculation. With
subpulmonary defects, it usually inserts
into the ventriculoinfundibular fold, or into

the posterior part of the muscular
ventricular septum. The outlet septum is
always devoid of any vital structure, so it
can be resected, or used as a secure site for
anchorage of sutures. In contrast, the
ventriculoinfundibular fold is not a solid
bar of muscle. Care must be taken to avoid

extensive dissection or resection of this
structure, as the right coronary artery lies
within its fold.
Knowledge of the type of
interventricular communication, as in
isolated ventricular septal defects, will give
accurate guidance to the disposition of the
conduction tissue. Obstruction of the
subpulmonary outow tract requires the
same attention to detail as when dealing
with tetralogy of Fallot. Other cardiac
anomalies will have to be dealt with in the
context of the particular cardiac
conguration present.
It may not have passed unnoticed that
discussion thus far of the group of hearts
unied because of their double-outlet
ventriculoarterial connections has paid
little attention to bilateral conuses. This is
because, as explained in our introductory
comments, we do not take this feature as an
integral component of the group. To us,
the term double outlet logically pictures a
specic ventriculoarterial connection, and
that is what we have described. The
presence or absence of bilateral
infundibular musculature depends simply
on whether the ventriculoinfundibular fold
interposes completely between the hinges
of the atrioventricular and arterial valvar

leaets, or is attenuated at some point to
permit brous continuity between them.
Either arrangement can exist when the
ventriculoarterial connection is one of a
double outlet.
A double outlet from the left ventricle is
very much more rare than a double-outlet
right ventricle. It can present with similar
variations in the position of the arterial
trunks, and their relationship to the
interventricular communication, as we
have described for the double-outlet right
ventricle. As with the double-outlet right
ventricle, the double-outlet left ventricle
can be found with any atrioventricular
connections. The rules for the disposition
of the conduction tissue will thus change
accordingly.
COMMON ARTERIAL TRUNK

A common arterial trunk is one that
supplies the systemic, pulmonary, and
coronary arteries directly (Figure 8.109)50.
The phenotypical feature of the lesion,
however, is the commonality of the
ventriculoarterial junction, which is
guarded by a common arterial valve
(Figure 8.110)51. In this way, the anomaly
303
is distinguished from the other types of

ventriculoarterial connection producing a
single outlet from the heart, namely a
solitary aortic trunk with pulmonary
atresia, a solitary pulmonary trunk with
aortic atresia, or a solitary arterial trunk52.
The presence of the common truncal valve
also distinguishes the entity from hearts
with a large aortopulmonary window,
which can effectively produce a common
intrapericardial arterial component.
While the difference between a common
trunk and an aortopulmonary window is
anatomical, the distinction between
common and solitary arterial trunks is more
semantic. We dene an arterial trunk as
being solitary rather than common when it
is not possible to nd any evidence of
intrapericardial pulmonary arteries52.
Description in this fashion resolves the
controversy as to whether the pulmonary
arteries, had they been present, would have
arisen from the arterial trunk or from the
heart (Figure 8.111). In patients with a
solitary trunk as thus dened, it is the
arterial supply to the pulmonary circulation
that is the key to surgical treatment. Such
patients are best considered along with
tetralogy of Fallot with pulmonary atresia
(see page 236; Figure 7.159).
Systemic arteries
Pulmonary
arteries
Coronary arteries
Sup.
Left
Right
Inf.
Fig. 8.109 The specimen, shown in anatomical orientation,

illustrates the essence of a common arterial trunk. A solitary trunk
leaves the base of the heart and directly supplies the systemic,
pulmonary, and coronary arteries.
304
Sup.
Common arterial trunk
Left
Right
Inf.
Fig. 8.110 The specimen, photographed in anatomical

orientation, shows how the phenotypical feature of the common
arterial trunk is the common ventriculoarterial junction, guarded in
this heart by a valve with three leaets. The common trunk is
supported predominantly by the right ventricle, with a potentially
restrictive interventricular communication (star).
Right ventricle
Common arterial valve
Common
arterial
trunk
Aorta
Possible
origin
from heart
Fig. 8.111 The cartoon illustrates the dilemma in dening the
Possible
origin
from trunk
The anatomical features that underscore

the options for, and success of, surgical
treatment for a common arterial trunk are
its connection to the ventricular mass, the
state of the truncal valve, the morphology
of the interventricular communication, the
presence of associated anomalies, and the
arrangement of the systemic and
pulmonary arterial channels arising from
the common trunk. The lesion can exist
with any atrioventricular connection, but it
nature of an arterial trunk in the absence of the intrapericardial

pulmonary arteries (dotted channels). There is no way of knowing
whether the pulmonary arteries, had they existed, would have
originated directly from the heart (left-hand panel) or from the
arterial trunk itself (right-hand panel). Had they originated from
the heart, the patent arterial trunk would have been an aorta. Had
the trunk itself given rise to atretic pulmonary arteries initially,
however, it initially would have been a common trunk. In this
situation, therefore, it is better to describe the arterial trunk simply
as a solitary structure.
is very rare to nd other than concordant

ones. The trunk itself usually overrides the
ventricular septum, being more or less
equally committed to the right and left
ventricles (Figure 8.112), although it can
take origin predominantly (Figure 8.110)
or exclusively (Figure 8.113) from the
morphologically right ventricle. When the
trunk arises entirely from the right
ventricle, problems can be produced
should the interventricular communication
be restrictive. This problem does not occur

when the trunk is committed
predominantly or exclusively to the left
ventricle. Right ventricular origin,
however, is frequently associated with the
presence of an atrioventricular septal defect
and a common atrioventricular junction
(Figure 8.114)53. This adds to the surgical
complexity, because not only is the septal
defect more distant from the arterial trunk,
but the left ventricular component of the
305
Common
truncal valve
Right
ventricle
Left
ventricle
Fig. 8.112 The heart has been sectioned along its long axis, and is
shown in anatomical orientation, demonstrating how the common
arterial trunk (black brace) overrides the crest of the muscular
ventricular septum (star), in this instance being equally committed
to both ventricles.
Sup.
Left
Right
Inf.
Fig. 8.113 In this heart with a common arterial trunk, seen in
Restrictive interventricular communication
common atrioventricular valve is trifoliate.

The need to secure a competent left valve
adds to the problems of surgical repair
signicantly.
anatomical orientation, the trunk arises exclusively from the right

ventricle. There is a completely muscular subtruncal infundibulum
(black dotted line). The interventricular communication is
restrictive. Should enlargement be attempted, the safe area for
resection is shown by the red dotted line.
The common ventriculoarterial

junction, which is the essence of the
common trunk, exists because of failure
of septation of the developing outow
tract54. Although the truncal valve can

be supported by a complete
infundibulum when it arises exclusively
from the right ventricle (Figure 8.113),
306
Bridging leaflets
Sup.
Right ventricle
Left
Right
Inf.
Fig. 8.114 The heart, photographed from the front, the right
ventricle having been opened, has a common arterial trunk
supported exclusively by the right ventricle. The interventricular
communication, however, is an atrioventricular septal defect. The
bridging leaets of the common atrioventricular valve are seen
extending into the left ventricle. The left atrioventricular valve was
trifoliate.
Origin of
pulmonary
arteries
Sup.
Left
Right
Inf.
Common
truncal
valve
Posterocaudal limb of SMT
more usually the interventricular

communication is directly juxta-arterial,
and there is typically brous continuity
between the leaets of the truncal and
Fig. 8.115 This heart, shown in anatomical orientation as seen

from the right ventricle, has a common arterial trunk overriding an
interventricular communication with a muscular posteroinferior
rim formed by fusion of the ventriculoinfundibular fold with the
posterocaudal limb of the septomarginal trabeculation (SMT). The
muscular rim protects the atrioventricular conduction axis. Note the
brous continuity between the leaets of the truncal and mitral
valves in the roof of the left ventricle (black dotted line).
mitral valves in the roof of the left

ventricle (Figure 8.115). When viewed
from the right ventricle, the septal defect
is cradled between the limbs of the
septomarginal trabeculation. In most

patients, the posterocaudal limb of the
trabeculation fuses with the
ventriculoinfundibular fold to produce a
307
Sup.
Common
truncal
valve
Left
Right
Inf.
Posterocaudal limb of SMT
muscular posteroinferior rim

(Figures 8.110, 8.112, 8.115). As with
other defects of this type, the muscular
structure thus formed serves to buttress
the axis of atrioventricular conduction
tissue from potential surgical damage.
More rarely, there can be brous
continuity between the truncal and
tricuspid valves. The defect is thus
perimembranous, and the conduction
axis is much more at risk during closure
when connecting the common trunk to
the left ventricle (Figure 8.116). Should it
be deemed necessary to enlarge a
restrictive interventricular
communication, this can be achieved by
resecting along its anterosuperior
margins (Figure 8.113). Sometimes, a
second muscular defect coexists with the
juxta-arterial defect. If present, such
defects must be identied and closed.
Cases have also been described in which
the ventricular septum has been
considered intact. This was because the
leaets of the truncal valve closed on the
septal crest during ventricular diastole55.
The juxta-arterial defect in this setting,
however, is readily evident when the
valvar leaets open during diastole. It is
possible to have a truly intact ventricular
Fig. 8.116 This heart with a common arterial trunk, viewed in

anatomical orientation, has an interventricular communication
(star) that is perimembranous, with brous continuity between the
leaets of the truncal and tricuspid valves (black dotted line),
because the ventriculoinfundibular fold stops short of the caudal
limb of the septomarginal trabeculation (SMT). There is also brous
continuity between the leaets of the tricuspid and mitral valves.
The atrioventricular conduction axis is at risk in the posteroinferior
rim of the defect.
septum should the arterial trunk arise

exclusively from the right ventricle, and
there is spontaneous closure of a preexisting interventricular communication.
The latter cases are unlikely to come to
surgical attention.
After surgical repair of a common trunk
following the Rastelli technique, the
truncal valve effectively becomes the aortic
valve. Valvar incompetence or stenosis, if
present, is therefore of considerable
signicance. This typically reects
dysplasia of the leaets (Figure 8.117). The
truncal valve itself usually has three leaets
(Figure 8.118). Quadrifoliate valves are also
relatively frequent, although the leaets are
often of unequal size (Figure 8.119). In
such circumstances, the surgeon may
choose to convert the valve into a trifoliate
pattern56. Valves with two leaets are also
found.
There are two classications currently
in use for the description of the variations
to be found in the arrangement of the
pulmonary and systemic channels taking
origin from the common trunk. Both
employ procrustean numerical
categorisations. The classical study of
Collett and Edwards50 was based on the
mode of origin of the pulmonary arteries.
The possibilities initially described were

for the arteries to arise from a short
conuent channel, to arise separately and
directly from the left posterior aspect of the
trunk, or to arise separately from either side
of the trunk. The initial classication also
included a fourth category, but those tting
in this grouping are recognised as
representing examples of tetralogy of Fallot
with pulmonary atresia, with pulmonary
arterial supply via systemic-to-pulmonary
collateral arteries. The second classication
was proposed by Van Praagh and Van
Praagh57. They initially divided the cases
into groups with or without an
interventricular communication. As we
have discussed, it is exceedingly rare to nd
patients having a common arterial trunk
and an intact ventricular septum; therefore,
it is their rst group that is of most
signicance. They also described four
subtypes, and grouped together the rst
two categorisations of Collett and Edwards,
retaining the third grouping as their second
type. The importance of their approach
was that it highlighted cases with
obstruction of the systemic circulation,
identied as a fourth grouping, and also
emphasised the possibility for a separate
origin of discontinuous pulmonary arteries,
308
Sinusal origin of pulmonary arteries
Sup.
Fig. 8.117 In this heart, the common arterial trunk has been
Ant.
Post.
Inf.
Dysplastic quadrifoliate valve
opened from the right side, and is shown in anatomical orientation.

The pulmonary arteries arise from within a truncal valvar sinus.
Note the origin of the left coronary artery from within the same
sinus. The truncal valve is dysplastic, and has four leaets.
Separate origin of pulmonary arteries
Left coronary
artery
Post.
Left
Right
Fig. 8.118 The common arterial trunk has been windowed from
Trifoliate truncal valve
Ant.
with one fed by a persistently patent arterial

duct, which could then become
ligamentous, these examples making up
their third group. Van Praagh and Van
the front, showing its posterior wall, from which the right and left
pulmonary arteries take separate origin. The truncal valve has three
leaets. Note the high origin of the left coronary artery.
Praagh57, however, also pointed out that

the overall collection of patients with a
common arterial trunk could be divided
into those with aortic as opposed to
pulmonary dominance within the trunk

itself. It is this notion of aortic as opposed to
pulmonary dominance that simplies
categorisation and description51,
309

Artery to
sinus node
Post.
Left
Right
Ant.
Fig. 8.119 In this example of a common arterial trunk, windowed

and opened from the right side, the truncal valve has four leaets,
but one is much smaller than the others (arrow). The artery to the
sinus node takes origin from the right coronary artery (star). Note
the high origin of the left coronary artery.
Adjacent origin of pulmonary arteries
Fig. 8.120 In this example of a common arterial trunk, opened

through the right ventricle from the front, the pulmonary arteries
take origin in adjacent fashion from the leftwards and posterior
part of an aortic dominant trunk. The interventricular
communication (star) has a muscular posteroinferior rim (red
double-headed arrow).
recognising that on rare occasions it may be

possible to nd patients in which the
intrapericardial common trunk divides into
aortic and pulmonary components of
comparable dimensions. In most instances,

it is the aortic component of the trunk that
is dominant. The pulmonary arteries
usually arise adjacent to one another from
the leftwards component of the trunk

(Figures 8.120, 8.121). If there is a
conuent pulmonary component, it is
usually very short (Figures 8.122, 8.123).
310
Post.
Left
Right
Ant.
RPA
LPA
Fig. 8.121 The computed tomographic angiogram, viewed from

above, shows how, in the setting of common arterial trunks, the
pulmonary arteries usually arise adjacent to one another from the
leftwards and posterior component of the trunk (white doubleheaded arrow). Note the crossing of the left pulmonary artery (LPA)
over the origin of the right pulmonary artery (RPA). This is
exaggerated when the pulmonary arteries take a more anterior
origin (see Figure 8.124).
Confluent origin of
pulmonary arteries
Left coronary
artery
Fig. 8.122 In this example of a common arterial trunk, the

pulmonary arteries take origin from a small conuent component
arising from the leftwards and posterior part of the common trunk,
which itself predominantly supplies the systemic circulation. The
interventricular communication (star) is perimembranous, as there
is brous continuity between the leaets of the truncal and
tricuspid valves (black dotted line), reinforced by a membranous
ap (arrow).
The pulmonary arteries can arise more

anteriorly, and are typically crossed at their
origins (Figure 8.124). More rarely, the
pulmonary arteries can arise within a
truncal valvar sinus (Figure 8.117)58, or can
take separate origins from the posterior

component of the dominant aortic trunk
(Figure 8.118). All of these patterns
facilitate connection to a right ventricular
conduit during complete repair. The less
common pulmonary dominant trunk is

found when the aortic component of the
trunk is hypoplastic, in association with
either coarctation or interruption of the
aortic arch (Figures 8.125, 8.126). It is in
311
RPA
LPA
Post.
Left
Right
Ant.
Fig. 8.123 The section comes from the computed tomographic

dataset prepared from the patient shown in Figure 8.121. There is a
very short conuent segment (black double-headed arrow) feeding
the origin of the right and left pulmonary arteries (RPA, LPA) from
the common trunk.
Aortic dominant trunk
Sup.
Left
Right
Fig. 8.124 In this heart with a common arterial trunk, the

pulmonary arteries arise from the anterior part of the trunk, which
is aortic dominant. The left and right pulmonary arteries cross as
they extend towards the hilums of the lungs (red arrows).
Inf.
this arrangement that the pulmonary

arteries arise from the sides of the trunk,
which continues through a persistently
patent arterial duct to feed the descending
aorta51. On occasion, however, the
pulmonary arteries can be crossed when

there is pulmonary dominance
(Figure 8.127). The cases with pulmonary
dominance produce greater problems for
surgical correction. Apart from these
cases, or when the pulmonary arteries

are discontinuous, with one fed via an
arterial duct, it is rare to nd persistence
of the duct. It is in the instances where
there is persistence of the duct that the
312
LSCA
RSCA
Arterial duct
LPA
RPA
LCCA
RCCA
Fig. 8.125 In this heart, the pulmonary component of the

common arterial trunk is dominant (red double-headed arrow). It
gives rise to the arterial duct, and to the right and left pulmonary
arteries (RPA, LPA), the latter arising from the sides of the
pulmonary component. The aortic component of the trunk is
grossly hypoplastic, supplying the right common carotid artery
(RCCA), but then interrupted beyond the origin of the left common
carotid artery (LCCA). There is a retro-oesophageal origin of the
right subclavian artery (RSCA), which arises from the descending
aorta along with the left subclavian artery (LSCA), the descending
aorta itself being fed by the arterial duct.
Hypoplastic aortic
component
Sup.
Stented arterial duct

Left
Right
Inf.
Hypoplastic aortic
component
Fig. 8.126 The computed tomographic angiogram, reconstructed

and seen from the front, shows another example of a pulmonary
dominant common arterial trunk. In this patient, the aortic arch
was interrupted at the isthmus. A stent has been placed in the
arterial duct, which supplies the descending aorta.
313
Stented arterial duct
Sup.
Right
Left
Inf.
systemic and pulmonary components are

more balanced as they divide from the
common trunk within the pericardial
cavity.
The surgeon should also take care to
identify the origins of the coronary
arteries59. When the truncal valve has four
sinuses, then the arteries typically arise
from the right- and left-sided sinuses.
There is less uniformity when, as is most
frequently the case, the truncal root has
three sinuses. It is unusual, nonetheless, for
coronary arteries to arise from the anterior
sinus60. Solitary coronary arteries are
frequent59. When there are two coronary
arteries, the left coronary artery often takes
an origin above the sinutubular junction,
which on occasion can be very high, with
transmural passage through the truncal
wall (Figure 8.128). The left coronary
artery can also be closely related to the
origin of the pulmonary arteries,
particularly when the pulmonary arteries
themselves take a truncal origin
(Figure 8.117). In all these settings, the
coronary arteries are at risk if unrecognised
when the surgeon separates the pulmonary
arteries from the common trunk.
Fig. 8.127 The dataset from the reconstruction of the computed

tomographic angiogram shown in Figure 8.126 is viewed from
behind. It shows crossing of the pulmonary arteries as they arise
from the posterior aspect of the common trunk. Note the right and
left pulmonary arteries that have been banded (white arrows), and
the stented arterial duct, which feeds the descending aorta in the
setting of the interrupted aortic arch.
Concordant ventriculoarterial
connections with parallel arterial
trunks
Hearts with this combination do not
necessarily have abnormal segmental
combinations, because if the
concordant, the segmental junctions will be
normal despite the unexpected
relationships of the arterial trunks. It is also
the case that patients with this arrangement
will be exceedingly rare, and it is possible
that the unusual position of the aorta could
go unnoticed. The same arrangement at the
ventriculoarterial junctions, nonetheless,
can be found with all the other possibilities
for atrioventricular connections; thus it is
appropriate to consider the combinations in
a separate section of this chapter, the more
so as they have been remarkably difcult to
understand. They are also currently
described with the most arcane of
terminologies by some, including such
spectacular phrases as isolated ventricular
inversion, and anatomically corrected
malposition. The essence of understanding
is to appreciate that, although the arterial
trunks arise from the morphologically

appropriate ventricles, they extend towards
the mediastinum in parallel rather than
spiralling fashion61,62. It is necessary,
therefore, to appreciate that the direction of
spiralling should be related to the topology
of the ventricular mass, and not the
position of the atrial chambers, the latter
being the approach taken by those who
produce terms such as isolated ventricular
inversion.
Thus, in the normal heart, the
pulmonary trunk, as it emerges from the
right ventricle, spirals in counter-clockwise
fashion around the aortic root as the
pulmonary arteries extend into the hilums
(Figure 8.129). In the mirror-imaged
arrangement, when there is left-hand
ventricular topology, the pulmonary
trunk spirals around the aortic root in
clockwise fashion (Figure 8.130). In the
arrangement of parallel trunks found with
concordant atrioventricular and
ventriculoarterial connections and the usual
atrial arrangement, the aorta arises from the
ventricular mass in a left-sided and anterior
position (Figure 8.131). In most instances,
the aortic valve is also supported within the
314
Main stem of left coronary artery
Sup.
Post.
Ant.
Inf.
RPA
Common
arterial
trunk
LPA
Circumflex artery
Fig. 8.128 The common arterial trunk is photographed from the

left side to show a very high origin of the left coronary artery, which
extends in intramural fashion as it exits the aortic dominant
component of the trunk. Note the crossed origin of the right (RPA)
and left (LPA) pulmonary arteries (compare with Figure 8.124).
Pulmonary trunk
Aorta
Sup.
Left
Right
Inf.
left ventricle by a complete muscular

infundibulum (Figure 8.132). It is not
essential, however, for there to be
discontinuity between the leaets of the
Fig. 8.129 The base of the normal heart is photographed to show

Right ventricle
the normal spiralling of the arterial trunks, with the pulmonary

trunk extending in counter-clockwise fashion around the aortic
root (black arrows).
aortic and the atrioventricular valves. It is

the insistence of the presence of bilateral
infundibulums that creates the need to
invent the arcane terms like isolated
ventricular inversion. We can illustrate the

simplicity of recognising parallel but
concordantly connected arterial trunks by
reference to the arrangement as seen in the
315
Sup.
Left
Right
Inf.
Aorta
Pulmonary trunk
Fig. 8.130 This heart is from a normal individual with mirrorimage arrangement of all the organs. The ventricular mass shows
left-hand topology, and the pulmonary trunk spirals in clockwise
fashion relative to the aortic root as it extends into the
mediastinum (black arrows).
Morphologically right ventricle
Pulmonary trunk
Juxtaposed atrial appendages
Aorta

Sup.
Left
Right
Inf.
presence of a double-inlet left ventricle.

In the most common variant of a doubleinlet left ventricle with concordant
ventriculoarterial connections, the lesion
known as the Holmes heart, the pulmonary
Fig. 8.131 This heart is from a patient with concordant

atrioventricular and ventriculoarterial connections. The aorta,
however, is left-sided and anterior relative to the pulmonary trunk.
Note the left-sided juxtaposition of the atrial appendages.
trunk, arising from the incomplete right

ventricle, spirals around the aortic root in
counter-clockwise fashion, as in the normal
heart (Figure 8.133). In the variant with
concordant connections but parallel arterial
trunks, although rare, the incomplete right

ventricle is usually left-sided. The
pulmonary trunk is thus also left-sided,
but enters the mediastinum in parallel
fashion to the arterial trunk, rather than
316
Sup.
Aorta
Left
Right
Inf.
Fig. 8.132 The left side of the heart demonstrated in Figure 8.131
Mitral valve
is shown, opened from its left side. The morphologically left atrium
is connected to a left ventricle with right-hand ventricular
topology. The aorta arises from the left ventricle, its valve
supported by an extensive muscular infundibulum (double-headed
arrow). There is also a large perimembranous ventricular septal
defect. Because the atrioventricular connections are concordant,
the atrioventricular conduction axis is posteroinferiorly positioned
(yellow dotted line).
Pulmonary trunk
Aorta
Ventricular
septal defect
Sup.
Left
Right
Incomplete morphologically
right ventricle
Inf.
spiralling as would be expected were there

normal but mirror-imaged relations
(Figure 8.134). Furthermore, the leaets of
the aortic valve, arising from the dominant
Fig. 8.133 The heart shown has a double-inlet left ventricle with
a right-sided incomplete right ventricle and concordant
ventriculoarterial connections, the lesion known as the Holmes
heart. The arterial trunks spiral in normal fashion, with the
pulmonary trunk extending in counter-clockwise fashion relative to
the aortic root (black arrows) (compare with Figure 8.129).
left ventricle, are usually in brous

continuity with the leaets of the
atrioventricular valves (Figure 8.135). A
similar arrangement can be found when the
incomplete right ventricle is right-sided, the

pulmonary trunk then entering the
mediastinum in a right-sided position
parallel to the aorta. These patterns can also
Sup.
317
Pulmonary trunk
Aorta
Post.
Ant.
Inf.
Ventricular
septal defect
Incomplete morphologically
right ventricle
Fig. 8.134 This heart exhibits a double-inlet left ventricle,

incomplete left ventricle, and concordant ventriculoarterial
connections. In this specimen, however, the incomplete right
ventricle is left-sided, and the pulmonary trunk arises in parallel
fashion, no longer spiralling around the aorta. This is an example of
concordant ventriculoarterial connections with parallel arterial
trunks.
Aorta
LAVV
RAVV

Sup.
Left
Right
Inf.
be found when there is absence of either the

right- or left-sided atrioventricular
connections and the left ventricle is
dominant. They should also be anticipated
Fig. 8.135 The dominant left ventricle from the heart shown in
Figure 8.134 is opened. The aortic valve arises from the ventricle
with its leaets in brous continuity (black dotted lines) with both
of the atrioventricular valves (RAVV, LAVV). Note the ventricular
septal defect communicating with the left-sided incomplete right
ventricle (arrow).
with a double-inlet right ventricle, although

we are not aware of any such descriptions.
Perhaps the most important subset of
patients with concordantly connected but
parallel arterial trunks are those with

discordant atrioventricular connections.
This arrangement is a close cousin to
congenitally corrected transposition, as
318
Perimembranous
VSD
Sup.
Pulmonary trunk
Post.
Ant.
Inf.
Fig. 8.136 The left-sided ventricle is photographed in a heart

Tricuspid
valve
with the usual atrial arrangement and discordant atrioventricular

connections. There is left-hand ventricular topology, but the
ventricle supports the pulmonary trunk above a complete muscular
infundibulum. The pulmonary trunk exits the heart parallel to the
aorta. Note the perimembranous ventricular septal defect (VSD).
Sup.
Ant.
Post.
Aorta
Inf.
Fig. 8.137 The right-sided morphologically left ventricle is shown
Mitral valve
discussed earlier in this chapter. The

combination of discordant connections at
the atrioventricular junction, but
concordant ventriculoarterial connections,
produces the haemodynamics of
transposition. In addition, as in the usual
from the heart illustrated in Figure 8.136. The atrioventricular

connections are discordant, but the aorta arises from the
morphologically left ventricle, with infundibular musculature
interposed between the leaets of the aortic and mitral valves.
Because of the discordant atrioventricular connections, the
conduction axis is anterior to the arterial valve (red star), and
descends (red dotted line) cephalad relative to the
perimembranous ventricular septal defect (white star) . The
combination of discordant atrioventricular and concordant
ventriculoarterial connections produces the haemodynamics of
transposition, but correction with an atrial redirection procedure
will restore the morphologically left ventricle to a systemic role.
form of transposition, the aorta is typically

anterior and right-sided. It is important,
however, to recognise that there is lefthand topology (Figure 8.136) in this
setting, as the optimal therapeutic approach
is to perform an atrial redirection
procedure, thus restoring the

morphologically left ventricle to its
appropriate systemic role. It is also
important, of course, to recognise and deal
with any associated malformations, such as
a ventricular septal defect (Figure 8.137).
Because the atrioventricular connections

are discordant in this combination, the
conducting tissue will arise from an
anomalous anterolateral node, and descend
anterocephalad relative to the expected
perimembranous defect (Figure 8.137). In
such hearts, there may be a subaortic
infundibulum, but the presence of brous
and mitral valves in the roof of the
morphologically left ventricle in no way
alters the diagnosis. Indeed, the hearts with
concordant ventriculoarterial connections
but parallel arterial trunks are exemplary in
serving to demonstrate the importance of
analysing, separately, the arterial
relationships and infundibular morphology
from the origin of the aterial trunks from
the ventricular mass.
10.
11.
12.
13.
14.
References cited
15.
1. Anderson RH, Macartney FJ, Tynan M,
et al. Univentricular atrioventricular
connection: the single ventricle trap
unsprung. Pediatr Cardiol 1983; 4:
273280.
2. Anderson RH, Becker AE, Tynan M, et al.
The univentricular atrioventricular
connection: getting to the root of a
thorny problem. Am J Cardiol 1984; 54:
822828.
3. Anderson RH, Ho SY. What is a ventricle?
Ann Thorac Surg 1998; 66: 616620.
4. Jacobs ML, Anderson RH. Nomenclature
of the functionally univentricular heart.
Cardiol Young 2006; 16(Suppl 1): 38.
5. Kurosawa H, Imai Y, Fukuchi S, et al.
Septation and Fontan repair of
univentricular atrioventricular connection.
314319.
6. Anderson RH, Baker EJ, Redington AN.
Can we describe structure as well as
function when accounting for the
arrangement of the ventricular mass?
Cardiol Young 2000; 10: 247260.
7. Franklin RCG, Spiegelhalter DJ, Anderson
RH, et al. Double-inlet ventricle presenting
in infancy. I. Survival without denitive
repair. J Thorac Cardiovasc Surg 1991; 101:
767776.
8. Gale AW, Danielson GK, McGoon DC,
Wallace RB, Mair DD. Fontan procedure
for tricuspid atresia. Circulation 1980; 62:
9196.
9. Laks H, Williams WG, Hillenbrand WE,
et al. Results of right atrial to right
ventricular and right atrial to pulmonary
16.
17.
18.
19.
20.
21.
22.
artery conduits for complex congenital

heart disease. Ann Surg 1980; 192: 382389.
Fontan F, Kirklin JW, Fernandez G, et al.
Outcome after a perfect Fontan
operation. Circulation 1990; 81: 15201536.
Hosseinpour AR, Anderson RH, Ho SY.
The anatomy of the septal perforating
arteries in normal and congenitally
malformed hearts. J Thorac Cardiovasc Surg
2001; 121: 10461052.
Cheung HC, Lincoln C, Anderson RH,
et al. Options for surgical repair in hearts
with univentricular atrioventricular
connection and subaortic stenosis. J Thorac
Abbott ME. Unique Case of Congenital
Malformation of the Heart. Museum Notes.
McGill University, 1992; pp. 522525.
Stefanelli G, Kirklin JW, Naftel DC, et al.
Early and intermediate-term (10 year) results
of surgery for univentricular atrioventricular
connection (single ventricle). Am J Cardiol
1984; 54: 811821.
de Leval MR, Kilner P, Gewillig M, Bull C.
Total cavopulmonary connection: a logical
alternative to atriopulmonary connection
for complex Fontan operations. J Thorac
Marcelletti C, Corno A, Giannico S,
Marino B. Inferior vena cavapulmonary
artery extracardiac conduit: a new form of
right heart bypass. J Thorac Cardiovasc
Surg 1990; 100: 228232.
Anderson RH, Arnold R, Thaper MK,
Jones RS, Hamilton DI. Cardiac specialized
tissues in hearts with an apparently single
ventricular chamber (double inlet left
ventricle). Am J Cardiol 1974; 33: 95106.
Keeton BR, Macartney FJ, Hunter S, et al.
Univentricular heart of right ventricular
type with double or common inlet.
Circulation 1979; 59: 403411.
Essed CE, Ho SY, Hunter S, Anderson
RH. Atrioventricular conduction system in
univentricular heart of right ventricular
type with right-sided rudimentary
chamber. Thorax 1980; 35: 123127.
Kiraly L, Hubay M, Cook AC, Ho SY,
Anderson RH. Morphologic features of the
uniatrial but biventricular atrioventricular
connection. J Thorac Cardiovasc Surg 2007;
133: 229234.
Orie JD, Anderson C, Ettedgui J,
Zuberbuhler JR, Anderson RH.
Echocardiographic-morphologic
correlations in tricuspid atresia. J Am Coll
Cardiol 1995; 26: 750758.
Bull C, de Leval M, Stark J, Macartney F.
Use of a subpulmonary ventricular
chamber in the Fontan circulation. J Thorac
319
23. Rao PS, Jue KL, Isabel-Jones J, Ruttenberg

HD. Ebsteins malformation of the
tricuspid valve with atresia. Am J Cardiol
1973; 32: 10041009.
24. Noonan JA, Nadas AS. The hypoplastic
left heart syndrome. An analysis of 101 cases.
Pediatr Clin North Am 1958; 5: 10291056.
25. Norwood WI, Lang P, Hansen DD.
Physiologic repair of aortic atresia
hypoplastic left heart syndrome. N Engl
J Med 1983; 308: 2326.
26. Van Praagh R. Transposition of the great
arteries II. Transposition claried. Am
J Cardiol 1971; 28: 739741.
27. Van Mierop LHS. Transposition of the
great arteries. Clarication or further
confusion? Editorial. Am J Cardiol 1971; 28:
735738.
28. Jaggers JJ, Cameron DE, Herlong JR,
Ungerleider RM. Congenital Heart
Surgery Nomenclature and Database
Project: transposition of the great arteries.
Ann Thorac Surg 2000; 69(Suppl 4):
S205S235.
29. Isaacson R, Titus JL, Merideth J, Feldt
RH, McGoon DC. Apparent interruption
of atrial conduction pathways after surgical
repair of transposition of the great arteries.
Am J Cardiol 1972; 30: 533535.
30. Ullal RR, Anderson RH, Lincoln C.
Mustards operation modied to avoid
dysrhythmias and pulmonary and systemic
venous obstruction. J Thorac Cardiovasc
Surg 1979; 78: 431439.
31. Shaher RM, Puddu GC. Coronary arterial
anatomy in complete transposition of the
great arteries. Am J Cardiol 1966; 17:
355361.
32. Yacoub MH, Radley-Smith R. Anatomy of
the coronary arteries in transposition of the
great arteries and methods for their transfer
in anatomical correction. Thorax 1978; 33:
418424.
33. Gittenberger-de-Groot AC, Sauer U,
Oppenheimer-Dekker A, Quaegebeur J.
Coronary arterial anatomy in transposition
of the great arteries: a morphologic study.
34. Chiu IS, Anderson RH. Can we better
understand the known variations in
coronary arterial anatomy? Ann Thorac Surg
2012; 94: 17511760.
35. Massoudy P, Baltalarli A, de Leval MR,
et al. Anatomic variability in coronary
arterial distribution with regard to the
arterial switch procedure. Circulation 2002;
106: 19801984.
36. Anderson RH, Becker AE, Arnold R,
Wilkinson JL. The conducting tissues in
congenitally corrected transposition.
Circulation 1974; 50: 911923.
320
37. Van Praagh R. What is congenitally

corrected transposition? N Engl J Med
1970; 282: 10971098.
38. de Leval M, Bastos P, Stark J, et al. Surgical
technique to reduce the risks of heart block
following closure of ventricular septal
defect in atrioventricular discordance.
515526.
39. Anderson RH, Becker AE, Gerlis LM. The
pulmonary outow tract in classically
corrected transposition. J Thorac
40. Becker AE, Ho SY, Caruso G, Milo S,
Anderson RH. Straddling right
atrioventricular valves in atrioventricular
discordance. Circulation 1980; 61:
11331141.
41. Ilbawi MN, DeLeon SY, Backer CL, et al.
An alternative approach to the surgical
management for physiologically corrected
transposition with ventricular septal defect
and pulmonary stenosis or atresia. J Thorac
42. Imai Y, Sawatori K, Hoshino S, et al.
Ventricular function after anatomic repair
in patients with atrioventricular
discordance. J Thorac Cardiovasc Surg
1994; 107: 12721283.
43. Wilcox BR, Ho SY, Macartney FJ, et al.
Surgical anatomy of double-outlet right
ventricle with situs solitus and
atrioventricular concordance. J Thorac
44. Walters HL 3rd, Mavroudis C,
Tchervenkov CI, et al. Congenital Heart
Surgery Nomenclature and Database
Project: double outlet right ventricle. Ann
Thorac Surg 2000; 69(Suppl 4): S249S263.
45. Lev M, Bharati S, Meng CCL, et al. A
concept of double-outlet right ventricle.
46.
47.
48.
49.
50.
51.
52.
53.

271281.
Stellin G, Zuberbuhler JR, Anderson, RH,
Siewers RD. The surgical anatomy of the
TaussigBing malformation. J Thorac
Stellin G, Ho SY, Anderson RH,
Zuberbuhler JR, Siewers RD. The surgical
anatomy of double-outlet right ventricle
with concordant atrioventricular
connection and non-committed ventricular
septal defect. J Thorac Cardiovasc Surg
1991; 102: 849855.
Lacour-Gayet F, Haun C, Ntalakoura K,
et al. Biventricular repair of double outlet
right ventricle with non-committed
ventricular septal defect (VSD) by VSD
rerouting to the pulmonary artery and
arterial switch. Eur J Cardiothorac Surg
2002; 21: 10421048.
Hosseinpour A-R, Jones TJ, Barron DJ,
Brawn WJ, Anderson RH. An
appreciation of the structural variability
in the components of the ventricular
outlets in congenitally malformed hearts. Eur
J Cardiothorac Surg 2007; 31: 888893.
Collett RW, Edwards JE. Persistent
truncus arteriosus. A classication
according to anatomic types. Surg Clin
North Am 1949; 29: 12451270.
Russell HM, Jacobs ML, Anderson RH,
et al. A simplied categorization for
common arterial trunk. J Thorac Cardiovasc
Surg 2011; 141: 645653.
Anderson RH, Thiene G. Categorization
and description of hearts with common
arterial trunk. Eur J Cardiothorac Surg
1989; 3: 481487.
Adachi I, Ho SY, Bartelings MM, et al.
Common arterial trunk with
atrioventricular septal defect: new
54.
55.
56.
57.
58.
59.
60.
61.
62.
observations pertinent to repair. Ann

Thorac Surg 2009; 87: 14951499.
Van Mierop LHS, Patterson DF, Schnarr
WR. Pathogenesis of persistent truncus
arteriosus in light of observations made in a
dog embryo with the anomaly. Am J Cardiol
1978; 41: 755762.
Carr I, Bharati S, Kusnoor VS, Lev M.
Truncus arteriosus communis with intact
ventricular septum. Br Heart J 1979; 42:
97102.
Backer CL. Techniques for repairing the
aortic and truncal valves. Cardiol Young
2005; 15(Suppl 1): 125131.
Van Praagh R, Van Praagh S. The anatomy of
common aorticopulmonary trunk (truncus
arteriosus communis) and its embryologic
implications. A study of 57 necropsy cases.
Am J Cardiol 1965; 16: 406426.
Adachi I, Uemura H, McCarthy KP, Seale
A, Ho SY. Relationship between orices of
pulmonary and coronary arteries in
common arterial trunk. Eur J Cardiothorac
Surg 2009; 35: 594599.
Suzuki A, Ho SY, Anderson RH, Deaneld
JE. Coronary arterial and sinusal anatomy
in hearts with a common arterial trunk. Ann
Thorac Surg 1989; 48: 792797.
Bogers AJ, Bartelings MM, Bokenkamp R,
et al. Common arterial trunk, uncommon
coronary arterial anatomy. J Thorac
Cardiovasc Surg 1993; 106: 11331137.
Bernasconi A, Cavalle-Garrido T, Perrin
DG, Anderson RH. What is anatomically
corrected malposition? Cardiol Young 2007;
17: 2634.
Cavalle-Garrido T, Bernasconi A, Perrin
D, Anderson RH. Hearts with concordant
ventriculoarterial connections but
parallel arterial trunks. Heart 2007;
93:100106.
Abnormalities of the
great vessels
322
Abnormalities of the right

superior caval vein
ANOMALOUS SYSTEMIC
VENOUS DRAINAGE
Abnormal systemic venous connections
are usually of little surgical signicance,
as their clinical consequences are limited.
The anomalies are apt to be encountered
as the surgeon pursues a more complex
associated intracardiac anomaly. They
are of most signicance in the setting of
isomeric atrial appendages, which we
discuss in Chapter 10, showing how socalled visceral heterotaxy is best
considered in terms of right versus left
isomerism. In this chapter, we consider
the features of the anomalous systemic
venous connections in their own right.
They may be grouped into the categories
of absence or abnormal drainage of the
right superior caval vein, anomalies of the
inferior caval vein, persistence or
abnormal drainage of the left superior
caval vein, and abnormal hepatic venous
connections. Abnormalities of the
coronary sinus usually fall into one of
these groups, although unroong,
which produces an interatrial
communication through the right atrial
orice of the sinus, has been discussed
in Chapter 7.
These are extremely rare. The vein may be

diminished in size. Alternatively, it may be
completely absent when the venous return
from the head, neck, and arms passes
through a persistent left superior caval vein
to the right atrium by way of the coronary
sinus (Figure 9.1) or, rarely, directly into
the left atrium. Only this last situation
requires surgical intervention. The other
conditions, if encountered during an
open-heart operation, would require some
adjustment from the usual technique used
for cannulation. Although there is no
denite evidence to this effect, we would
not expect these abnormalities to affect the
location of the sinus node.
Abnormalities of the inferior

caval vein
The inferior caval vein has been described to
connect directly to the morphologically left
atrium1, producing right-to-left shunting,
although we have never seen such a lesion.
When the inferior caval vein does drain to
the left atrium, this is usually because a
persistent Eustachian valve directs ow from
the right atrium through the oval fossa
(Figure 9.2). Such right-to-left shunting can

also be an iatrogenic phenomenon,
occasionally observed when low-lying atrial
septal defects are improperly closed (see
Chapter 7). The more frequent anomaly
involving the inferior caval vein is
interruption of its terminal segment, with
return of the venous blood from the lower
body through the azygos or hemiazygos
venous systems (Figure 9.3)2. The
malformation is seen most frequently with
isomerism of the left atrial appendages3. The
hepatic veins usually drain independently
into the right-sided or left-sided atrium,
although there can be a conuent
suprahepatic channel, as is usually the case
when interruption and azygos continuation
is found with usual or mirror-imaged atrial
chambers (Figure 9.4).
Persistence of the left superior

caval vein
Persistence of the left superior caval vein is
the most common malformation involving
the caval veins (Figure 9.1). In about
three-fths of reported cases4, the persisting
left vein is joined to the right superior caval
vein by a brachiocephalic vein. When
arranged in this fashion, the left caval vein
Left atrial
appendage
Pulmonary
trunk
Left
Sup.
Inf.
Right
Fig. 9.1 This operative view through a median sternotomy shows

a left superior caval vein entering the pericardial cavity between
the left atrial appendage and the pulmonary trunk.
Persisting Eustachian valve
can simply be clamped or ligated, so as to

avoid ooding the eld when the heart is
opened. If connections with the right vein are
not apparent, a trial period of occlusion will
usually indicate whether venous
hypertension will give problems. A left
superior caval vein is found not infrequently
in patients with cyanotic heart disease, being
reported in up to one-fth of patients with
tetralogy of Fallot, and one-twelfth of
patients with Eisenmengers syndrome5. The
venous channel can be the route of partially
or totally anomalous pulmonary venous
connection. It can also empty directly into the
left atrium, as in the constellation described
as unroong of the coronary sinus (see
Chapter 7). Much more frequently, the vein
persists as an isolated venous channel, which
usually receives the hemiazygos vein before
penetrating the pericardium and passing
between the left atrial appendage and the left
pulmonary veins (Figure 9.5). It then
323
Fig. 9.2 The cartoon shows the most frequent mechanism by

which the venous return through the inferior caval vein drains to
the left atrium. It is deected across a defect within the oval fossa
(red arrow) due to persistence of the Eustachian valve.
connects with the coronary sinus, traversing

the inferior left atrioventricular groove to
empty into the right atrium through a larger
than normal orice (Figure 9.6). In such
hearts, the Thebesian valve is often
attenuated or absent. There are also reports
of the persistent vein obstructing ow from
the pulmonary veins towards the atrial
vestibule6. It is certainly the case that the
venous channel could bulge into the cavity of
the left atrium.
veins are normally connected to the

morphologically left atrium, the pattern of
venous return is comparable to the
snowman types of anomalous pulmonary
venous connection (see later). A variant of
the levoatrial cardinal vein is found when
there is atresia of the right atrial orice of the
coronary sinus (Figure 9.9). A vertical vein
can drain the coronary venous blood to the
brachiocephalic vein and thence to the right
atrium via the superior caval vein
(Figure 9.10).
Levoatrial cardinal vein

Another anomaly that, although rare,
warrants consideration is the levoatrial
cardinal vein7,8. This channel connects the
left atrium to the systemic venous system. It
is found with associated lesions such as mitral
atresia, where it functions as the only route
for pulmonary venous return (Figures 9.7,
9.8). In this case, although the pulmonary
ANOMALOUS PULMONARY
VENOUS CONNECTION
Very rarely, the pulmonary veins may be
obstructed or totally atretic at their atrial
junction9. This is unlikely to be a surgically
remedial situation. Much more commonly,
the pulmonary venous system, either
totally or in part, has an anomalous
324
Sup.
Left
Right bronchus
Right
Inf.
Aorta
Fig. 9.3 The heart is photographed from behind, showing the
Hepatic veins to right atrium
venous return from the abdomen returned to the heart through

the azygos system of veins (arrows). The inferior caval vein is
interrupted below the diaphragm, and returns only the blood
received through the hepatic veins.
Azygos vein
Superior caval vein
Morph. right
appendage
Fig. 9.4 This heart, photographed from the right side in
Sup.
Post.
Ant.
Inf.
Hepatic veins
anatomical orientation, shows the termination of the azygos vein,

which is returning the blood from below the diaphragm to the
superior caval vein in the setting of interruption of the inferior
caval vein (see Figure 9.3). Note the structure of the
morphologically (Morph.) right appendage, indicating the
presence of the usual atrial arrangement.
Left pulmonary
veins
325
Left appendage
Fig. 9.5 The heart is photographed from above in anatomical

Sup.
Ant.
Post.
Inf.
Coronary sinus
orientation, the domes of both atrial chambers having been

removed. A persistent left superior caval vein enters the left
atrioventricular groove between the left appendage and the left
pulmonary veins, with its course through the groove marked by the
red dotted lines. It enters the right atrium through the enlarged
coronary sinus (see Figure 9.6.) Note that, as it passes through the
left atrioventricular groove, it potentially obstructs pulmonary
venous ow to the mitral valvar vestibule.
SCV
Oval fossa
ICV
Sup.
Ant.
Post.
Enlarged mouth of
coronary sinus
Inf.
Fig. 9.6 This photograph in anatomical orientation shows the

enlarged orice of the coronary sinus within the right atrium of a
heart with a persistent left superior caval vein. This is not the same
heart as is shown in Figure 9.5. SCV, (right) superior caval vein; ICV,
inferior caval vein.
326
Azygos vein
SCV
Anomalous vein
Left atrium
Morph. right
appendage
Sup.
Ant.
Post.
Pulmonary veins
Inf.
Fig. 9.7 This specimen, from a patient with hypoplasia of the left
heart, photographed in anatomical orientation, shows the course
of the so-called levoatrial cardinal vein. The pulmonary veins
connect in normal fashion to the left atrium, but the anomalous
vein drains the atrial contents to the superior caval vein (SCV). Thus,
there are normal venous connections, but anomalous drainage
(arrows).
Sup.
BCV
Left
Right
Inf.
Anomalous vein
Left atrium
Fig. 9.8 The reconstructed dataset prepared from a computed

tomographic angiogram shows a levoatrial cardinal vein extending
from the right lower pulmonary vein and taking a tortuous course
before draining into the brachiocephalic vein (BCV). The patient
had coexisting mitral atresia and an intact atrial septum.
Pulmonary veins
connection. There is a plethora of

possibilities for abnormal pulmonary
venous connections10. The arrangements
should be described as specically and as
unambiguously as possible (Figure 9.11).
The pulmonary veins connect anomalously
when they are not joined to the
morphologically left atrium. Consequently,

if all the veins from one lung connect to a
site other than the left atrium, the
arrangement can be described as a
unilateral totally anomalous pulmonary
venous connection. If all the veins from
both lungs connect to sites other than the
morphologically left atrium, there is a

totally anomalous pulmonary venous
connection. This, of necessity, implies a
bilateral totally anomalous connection.
Any combination can readily be
described by treating each lung as an entity.
The unifying feature in all of these
327
Sup.
Post.
Ant.
Inf.
Tricuspid
valve
Oval
fossa
Atretic mouth of
coronary sinus
Fig. 9.9 In this heart, there is atresia of the mouth of the

coronary sinus. The coronary venous blood returns through a
vertical vein, as shown in Figure 9.10.
Vertical vein
Sup.
Ant.
Fig. 9.10 The heart shown in Figure 9.9 is photographed from

Post.
Coronary sinus
Inf.
combinations is the connection of one or

more pulmonary veins to the systemic
venous system or to the right atrium. It is
equally important, therefore, that the site of
behind to show the vertical vein that drains the coronary veins
(arrows), themselves connected to the obstructed coronary sinus
(see Figure 9.9), to the left brachiocephalic vein, and thence to the
superior caval vein. This situation is analogous to the levoatrial
cardinal vein providing pulmonary venous egress in the setting of
an obstructed left atrium.
drainage be identied. Historically, this has

been described as supradiaphragmatic or
infradiaphragmatic (Figure 9.12).
Supradiaphragmatic drainage can further
be subdivided into supracardiac and

cardiac patterns. Supracardiac connection
may be to a right or left superior caval vein,
the brachiocephalic or innominate vein, or
328
Partially anomalous
unilateral drainage
Fig. 9.11 This cartoon, in anatomical

Totally anomalous
bilateral drainage
Totally anomalous unilateral drainage
orientation, shows how an anomalous

connection of the pulmonary veins can be well
accounted for by describing the connection of
the veins from each lung as separate entities,
and then describing partially as opposed to
totally anomalous drainage on each side. The
site of drainage needs to be specied
separately (see Figure 9.12).
Supracardiac connection
to superior caval vein
to azygos vein
Cardiac connection
to LSCV & coronary sinus
direct to right atrium
Infradiaphragmatic &
infracardiac connection
to portal venous system
to inferior caval vein
even the azygos vein. Connection to the

brachiocephalic vein is most frequent. The
combination of drainage via a left-sided
vertical vein, and thence via the
brachiocephalic vein to the superior caval
vein (Figures 9.13, 9.14), produces the
typical snowman conguration as seen on
chest radiography. The drainage can be
Fig. 9.12 This cartoon, in anatomical orientation, shows the

potential sites for an anomalous connection of the pulmonary
veins. LSCV, left superior caval vein.
obstructed when the vertical vein crosses in

front of the left bronchus, but obstruction
is more usually produced when the venous
channel is caught in a vice between the left
pulmonary artery and the left bronchus
(Figure 9.15). When the route of drainage
involves the azygos vein, the left
pulmonary veins tend to cross vertically
beneath the heart, ascending in the right

paravertebral gutter and receiving the right
pulmonary veins, before draining to the
superior caval vein via the azygos vein
(Figure 9.16). More bizarre patterns of
drainage can occur when the right-sided
veins cross to the left, join with the
left-sided veins, and make their connection
329
SCV
Ascending vein
Ventricular mass
Fig. 9.13 This heart, photographed from the front in anatomical

orientation, has a totally anomalous pulmonary venous connection
to the superior caval vein (SCV). With this arrangement, the
heart forms the body of the snowman seen in the chest
radiograph, with the anomalous vein forming the head of the
snowman as it courses to drain to the superior caval vein (arrow).
Sup.
Right
Left
Inf.
Ascending vein
Pulmonary venous confluence

Ventricular mass

tomographic angiogram shows a supracardiac totally anomalous
pulmonary venous drainage as viewed from behind. The
brachiocephalic vein drains to the superior caval vein.
330
Sup.
Right
Left
Inf.
Fig. 9.15 In this specimen, from a patient with a totally
Left bronchus
anomalous pulmonary venous connection, the mediastinum

is photographed from the front, the heart having been
reected from its pericardial cradle. The draining channel from
the pulmonary venous conuence is obstructed in a
bronchopulmonary vice.
Morph. right appendage (left-sided)

LSCV
Fig. 9.16 This heart is from a patient with a mirror-imaged
Sup.
Right
Left
Inf.
to the azygos vein adjacent to the

diaphragm11.
Drainage directly to the right atrium is
typically through the coronary sinus
(Figure 9.17). In our experience, a totally
anomalous connection to the right atrium
other than through the coronary sinus has
arrangement of all the organs, including the heart. Note the

left-sided position of the morphologically (Morph.) right atrial
appendage. The heart has been reected from its pericardial
cradle, and the photograph taken from the front. The pulmonary
veins drain anomalously to the left-sided superior caval vein (LSCV),
with the collecting channel passing below the heart and
ascending within the left-sided paravertebral gutter. The stars
show the right pulmonary vein, while the arrow shows the left
upper pulmonary vein.
been seen only in the setting of isomerism of

the right atrial appendages (Figure 9.18).
The pulmonary veins, even when they
return to the heart, are always connected in
anatomically anomalous fashion when both
of the atrial appendages are of right
morphology. An infradiaphragmatic
connection is usually total except when there

is a partially right-sided connection to the
inferior caval vein. This latter arrangement
is the so-called scimitar syndrome, a name
taken from its likeness to a Turkish sword
when seen in the chest radiograph12. In the
usual infradiaphragmatic connection, the
Sup.
Ant.
331

Post.
Inf.
Left atrium
Fig. 9.17 This specimen, viewed from the left side in anatomical
Mouth of coronary sinus
Morphologically right appendage
Sup.
Post.
orientation, has an anomalous connection of all four pulmonary

veins to the coronary sinus, which drains to the right atrium.
Ant.
Inf.
Fig. 9.18 In this heart, all four pulmonary veins (arrows) are
Absence of coronary sinus
common channel connecting both lungs

lies outside the pericardium posterior to the
left atrium (Figure 9.19). A vertical vein
drains through the diaphragm as a single
channel to enter the portal vein or the
venous duct (Figures 9.20, 9.21). The return
from the lungs becomes obstructed
connected directly to the right atrium, the lowermost vein through

two tributaries. There was isomerism of the right atrial
appendages. Note the absence of the coronary sinus.
subsequent to closure of the venous duct.

Occasionally, the channel breaks up into a
series of branches that connect to the
gastric veins (Figure 9.22). It is very rare to
nd an infradiaphragmatic connection
directly to the inferior caval vein. The
totally anomalous pulmonary venous
connection can also drain to a mixed site. In

the image shown in Figure 9.23, the upper
pulmonary veins drain in supracardiac
fashion to the superior caval vein, while the
inferior pulmonary veins drain to the
coronary sinus. Any combination should be
anticipated.
332

Body of left atrium
Pulmonary venous
confluence
Sup.
Ant.
Fig. 9.19 This specimen, viewed from the left side in
Post.
anatomical orientation, has an anomalous connection of all

pulmonary veins to a descending channel (arrow) that passes
through the diaphragm to join the portal venous circulation.
Inf.
Pulmonary venous
confluence
Sup.
Left
Hepatic portal
vein
Right
Inf.
The salient anatomical features

relating to surgical repair of anomalous
pulmonary connections include the
type of abnormal connection, the site of

anomalous pulmonary venous connection, the mediastinum is
viewed from behind. The descending pulmonary venous channel
passes through the diaphragm and empties into the hepatic
portal vein.
the anomalous connection, and the

proximity of the anomalous veins to the
left atrium. As pointed out in Chapter 7,
an interatrial communication in the
mouth of the superior caval vein, the

so-called sinus venosus defect, is
always accompanied by an anomalous
connection of the right pulmonary
333
Pulmonary veins
Vertical vein
Sup.
Left
Right
Inf.

tomographic angiogram shows infracardiac and
infradiaphragmatic totally anomalous pulmonary venous
drainage as viewed from behind. The descending vertical vein
drains to the hepatic portal venous system.
Hepatic portal vein
Sup.
Confluence
Right
Left
Inf.
Descending
vein

Leash of
draining vessels
veins to the superior caval vein. The

need to safeguard the sinus node and
its blood supply has already been
emphasised.
anomalous pulmonary venous connection, the heart has been

reected from its pericardial cradle. The descending channel passes
through the diaphragm and fragments into a leash of small veins
that join the gastric veins.
Other sites of connection, or potential

sites of anastomosis, often require
construction of an extensive atrial junction
to guard postoperatively against pulmonary
venous obstruction. The appropriate

landmarks must be borne in mind. Two
types of anomalous connection pose
particular problems. The rst is when the
334
Sup.
Left
Right
Inf.
Left superior pulmonary vein
Coronary sinus
Left inferior and right

pulmonary veins
anomalous venous return is by way of the

coronary sinus (Figures 9.17, 9.23). We
initially thought that the myocardium
between the coronary sinus and the left
atrium was common to both structures. We
now know that the sinus has its own
myocardial wall, separate from that of the
left atrium13. It is possible, nonetheless, to
incise both walls so as to unroof the sinus.
At the same time, the orice of the coronary
sinus can be made conuent with the atrial
septal defect, which is typically within the
oval fossa. It is important to remove enough
of the walls to ensure that the patch placed
across the orice of the coronary sinus and
oval fossa does not produce obstruction at
the site of the incised sinus septum. Ideally,
the incised walls of the atrium and coronary
sinus should also be repaired, because the
incisions create a route to the inferior
atrioventricular groove. Thus far, however,
we are unaware of any reports of
tamponade following such procedures.
The surgeon should be aware, nonetheless,
that a potential opening is created to the
extracardiac spaces. When incising the
coronary sinus, it is also important to avoid
the atrioventricular node at the apex of the
triangle of Koch.
The second problematic type of
connection is the infradiaphragmatic
variant. The extrapericardial common
tomographic angiogram shows mixed totally anomalous

pulmonary venous drainage as viewed from behind. The left
superior pulmonary vein drains to the brachiocephalic vein, and
thence to the superior caval vein, while the left inferior and the
right pulmonary veins drain to the coronary sinus.
pulmonary venous trunk is further from

the posterior left atrial wall than might
be expected (Figure 9.19). Because of this,
the anastomosis constructed between the
trunk and left atrium is vulnerable to
obstruction, particularly at its lateral
extreme. This may become evident only
when bypass is discontinued and the heart
lls with blood.
ANOMALIES OF THE AORTA

The congenital anomalies of the thoracic
aorta that are of interest to the surgeon
include coarctation, the spectrum of partial
to complete interruption of the aorta, and
vascular rings and slings.
Aortic coarctation
Coarctation is a congenitally derived
discrete shelf-like (Figure 9.24) or waistlike (Figure 9.25) lesion within the aorta,
which causes obstruction to the ow of
blood. It is most often found adjacent to the
opening of the persistently patent arterial
duct (Figure 9.26), or the arterial ligament
if the duct has closed, but can occur
proximally or more distally, and even in the
abdominal aorta. It is usually accompanied
by some degree of tubular hypoplasia of the
aorta, but is anatomically independent of

the hypoplasia. It can coexist with other
lesions that diminish left-sided ow, but
coarctation can, and does, occur
independently. Tubular hypoplasia, when
involving the isthmic segment of the aortic
arch, can then be considered as part of a
spectrum leading to atresia or interruption
of the aortic arch.
Much has been made of the role of
ductal tissue in the aetiology of coarctation.
Although there was a time when some
doubted its signicance, the evidence
supporting its inclusion in the coarctation
shelf (Figure 9.24) is now unequivocal1416.
The surgeon, therefore, needs to be aware
of the importance of removing all the
ductal tissue during repair. The
signicance of the duct itself devolves upon
its patency. If the duct is patent, typically
there is an associated congenital anomaly
that promotes increased ow of blood
through the pulmonary trunk, and diverts
blood away from the proximal aorta. In
these circumstances, the associated
anomaly tends to dominate the picture, and
will determine the most appropriate
surgical therapy. On the other hand,
coarctation with a closed duct is very likely
to be an isolated lesion (Figure 9.25),
except for the occasional association with
a bifoliate aortic valve17.
335
Isthmal hypoplasia
Arterial duct
Coarctation shelf is
ductal tissue
Sup.
Ant.
Post.
Fig. 9.24 This specimen, viewed in anatomical orientation, shows

Inf.
Descending aorta
the ductal tissue that forms the shelf lesion of aortic coarctation.
Note the coexisting hypoplasia of the aortic isthmus.
Left subclavian
artery
Sup.
Ant.
Descending aorta
Post.
Inf.
The chief concerns of the surgeon relate

to the specic anatomy of the coarctation.
The nature of the collateral circulation is of
particular signicance. With welldeveloped collateral arteries (Figure 9.27),
there is little danger of cross-clamping the

tomographic angiogram shows a waist-like constriction (arrows)
between the aortic isthmus and the descending aorta. In this
patient, the arterial duct has closed.
aorta during repair. If the collateral arteries

are less well developed (Figure 9.28),
however, clamping the aorta may have
several deleterious consequences. These
include strain on the left ventricle due to
proximal hypertension, which may also
induce a cerebrovascular accident

secondary to rupture of a berry aneurysm.
There may be distal aortic hypotension,
at pressures of less than 50 mmHg, which
endangers the splanchnic and spinal
vascular beds. Irrespective of the nature
336
Sup.
Ant.
Left subclavian
artery
Post.
Inf.
Aortic
isthmus

tomographic angiogram shows a coarctation lesion (arrow) in a
juxtaductal position with a persistently patent arterial duct. In this
patient there is considerable length to the aortic isthmus (compare
with Figure 9.25).
Arterial duct
Left subclavian
artery
Recurrent laryngeal nerve
Coarctation at isthmus
Ant.
Inf.
Enlarged intercostal arteries
of the collateral arteries, the spinal

circulation is best preserved by
interrupting none, or as few as possible,
of the intercostal vessels. Temporary
Sup.
Fig. 9.27 This operative view, through a left thoracotomy,

Post.
shows aortic coarctation with enlarged intercostal arteries, part

of a well-developed collateral circulation.
occlusion of the intercostal vessels adjacent

to the operative eld seems to be a
reasonable compromise in this difcult
situation.
Other noteworthy features include the

position of the thoracic duct (Figure 9.29),
and the occasional presence of an anomalous
artery (Figure 9.30), sometimes referred to
Arterial ligament
Ant.
337
Aortic arch
Sup.
Inf.
Post.
Coarcted segment
Fig. 9.28 In this operative view through a left thoracotomy, from

Normally sized intercostal artery
a patient with aortic coarctation (compare with Figure 9.27), the

collateral circulation is not well developed.
Aorta
Ant.
Inf.
Sup.
Post.
Thoracic duct
as Abbotts artery. When present, this artery

arises from the posteromedial aspect of the
proximal descending aorta. Whether it is an
enlarged bronchial artery, or persistence of
the evanescent fth arch, as initially
suggested by Hamilton and Abbott18, is
irrelevant. What is important is that the
surgeon is aware of the existence of this
Fig. 9.29 This view through a left lateral thoracotomy shows the
thoracic duct as it passes from the area of coarctation behind the
left subclavian artery.
anomalous vessel that, if not properly

managed, can lead to substantial bleeding
during the repair19.
Interruption of the aortic arch

Discontinuity of the aorta, as opposed to
coarctation, has been variously referred to
as absence, atresia, or interruption of the

arch. Included in this group are those cases
with a brous cord or bridge of tissue
(Figure 9.31), as well as those with a gap, or
absolute discontinuity, at some point in the
arch (Figures 9.32, 9.33). Interruption can
occur at one of three positions: at the
isthmus, between the left subclavian and
338
Aortic isthmus
Descending aorta
Ant.
Inf.
Sup.
Post.
Abbotts artery
Intercostal arteries
Fig. 9.30 As seen through a left thoracotomy, the proximal

descending aorta and distal arch have been rotated anteriorly. The
large collateral vessel referred to as Abbotts artery is seen arising
proximally to the area of coarctation.
Atretic cord
Aorta
Sup.
Right
Fig. 9.31 This specimen, photographed in anatomical
Left
Duct to descending
aorta
Inf.
left common carotid arteries, and between

the left common carotid and
brachiocephalic arteries (Figure 9.34).
These lesions typically are found in the
setting of a unilateral left aortic arch but,
orientation, has atresia of the isthmus, with a thin brous cord

running from the site of effective interruption at the left subclavian
artery to the distal descending aorta, which is fed through the
arterial duct.
rarely, a right arch can be affected

similarly20. As patients with discontinuity
of the aorta are unlikely to survive without
surgical treatment, they are a particular
challenge to the clinician. The associated
cardiovascular malformations are of

critical importance, for they affect the
operative outcome as much as does an
interrupted arch itself. Almost always,
there is a patent connection to the distal
339
RSCA
LCCA
LSCA
RCCA
Duct
Sup.
Pulmonary
trunk
Aorta
Fig. 9.32 In this specimen, shown in anatomical orientation as

Right
photographed from the front, the aortic arch is interrupted (star) at

the isthmus, with a retro-oesophageal origin of the right subclavian
artery (arrow). The descending aorta is fed from the pulmonary
trunk via the arterial duct. LCCA and RCCA, left and right common
carotid arteries; LSCA and RSCA, left and right subclavian arteries.
Left
Inf.
Sup.
Ascending aorta
Pulmonary trunk
Left
Right
Inf.
Arterial duct
BCA
LCCA
Descending
aorta
tomographic angiogram shows interruption of the aortic arch

(white dotted double-headed arrow) in between the left common
carotid artery (LCCA) and the left subclavian artery (LSCA). The
hypoplastic ascending aorta also supplies the brachiocephalic
artery (BCA).
LSCA
aorta through the duct (Figure 9.33).

A proximal septal defect is also the rule.
This is most frequently a ventricular
septal defect but, occasionally, an
aortopulmonary window is found. The

ventricular septal defect typically is
associated with posterior and leftward
displacement of the outlet septum,
resulting in subaortic stenosis

(Figure 9.35). Any type of defect,
nonetheless, can be found. Abnormal
ventriculoarterial connections are not
340
RCCA
LCCA
RCCA
RCCA
LCCA
LSCA
LCCA
LSCA
LSCA
Fig. 9.34 This cartoon, in anatomical
Interruption
between
carotid arteries
(Type C)
Interruption
between
LCCA and LSCA
(Type B)
Interruption
at isthmus
(Type A)
orientation, shows the different sites of

interruption of the aortic arch. The ascending
aorta, and the vessels it feeds, are shown in red.
The pulmonary trunk, and the vessels fed via
the arterial duct, are shown in blue. The right
subclavian artery usually takes origin from
the brachiocephalic trunk, but can have a
retro-oesophageal origin from the descending
aorta (see Figure 9.32). LCCA and RCCA, left
and right common carotid arteries; LSCA, left
subclavian artery.
VSD with muscular post-inf. rim
Fig. 9.35 This view of a specimen sectioned to replicate the

Deviated outlet septum
Narrowed subaortic outlet
unusual, and present their own particular

problems for operative reconstruction21.
A less lethal but fairly frequently
associated arterial abnormality is the
aberrant origin of a retro-oesophageal right
subclavian artery from the distal aorta
(Figure 9.32). Because the arch is
interrupted, symptoms of a vascular ring
are unlikely, unless a ring is created while
reconstructing the arch. Conversely, if the
aberrant artery is brought forward, it can be
useful in repairing the aorta. This
parasternal echocardiographic cut, in the setting of interruption of

the aortic arch, seen in anatomical orientation, shows posterior
deviation of the outlet septum into the left ventricle, with
obstruction of the subaortic outow tract. The ventricular septal
defect (VSD) has a muscular posteroinferior (post-inf.) rim.
emphasises the importance of dening

clearly the nature and effect of associated
abnormalities. Successful anatomical
correction is as dependent on appropriate
management of these accompanying lesions
as it is on establishing aortic continuity.
Vascular rings
Aortic rings are malformations associated
with abnormal regression of part of a
double aortic arch. Knowledge of the
development of the aortic arches is a

genuine aid to understanding the
morphology of these anomalies. Initially,
the arterial system is bilaterally
symmetrical, with ve sets of arches
encircling the gut, and on occasion the
remnants of a fth set being evident for
short periods (Figure 9.36). Of these, only
the third, fourth, and sixth arches remain
recognisable in the postnatal heart.
Remnants of the third arch supply the
head. The left fourth arch becomes part of
13
13
4
6
Ao
341
Ao
PT
PT
Arterial duct
Left aortic arch
Fig. 9.36 This cartoon, in anatomical orientation, shows the

patterns of formation of the aortic arches believed to exist during
embryological development. The numbers refer to the arteries
supplying the pharyngeal arches of the developing embryo. The
transient fth aortic arches are shown by the red dotted lines. The
arrows show the seventh segmental arteries, which will become the
subclavian arteries. Initially they are distal to the insertion of the sixth
aortic arch. Ao, aorta; PT, pulmonary trunk.
R
SCA
CCA
Fig. 9.37 This cartoon shows how the denitive system of aortic
arches is derived from the embryological primordiums depicted in
Figure 9.36. Note that the subclavian arteries (arrows), derived from
the seventh segmental arteries, have migrated proximal to the
origin of the ducts, and that the right aortic arch has atrophied
(yellow and red dotted channels). The left sixth arch persists as the
arterial duct.
L
Oesophagus
SCA
Trachea
CCA
Duct
Duct
Fig. 9.38 The cartoon shows the perfect hypothetical double arch, with right (R) and left
Ao
(L) components, and with a subclavian artery (SCA), common carotid artery (CCA), and arterial
duct arising from each arch. The descending aorta (arrow) is in the midline, or in a neutral
position. Ao, aorta; PT, pulmonary trunk.
PT
the denite aortic arch. The left sixth arch

becomes the arterial duct (Figure 9.37).
The subclavian arteries, which are the
seventh cervical intersegmental arteries,
come to take origin from the fourth arch
between the third and sixth arches when
the heart migrates inferiorly22. Thus,
normally during early development, most
of the right-sided structures regress,
leaving a left arch and a left-sided aorta
(Figure 9.37). It is failure of this normal
regression that leaves the double arch,
which then forms a ring around the trachea

and the oesophagus. The so-called
hypothetical double aortic arch23 is a
concept that explains malformations
encircling the tracheo-oesophageal pedicle
on the basis of incomplete regression. The
hypothetical pattern comprises a midline
anterior aorta, continued anteriorly on each
side by the fourth arches to bilateral
posterior aortic segments, which join to
form a midline descending aorta. Common
carotid and subclavian arteries arise from
each fourth arch in the hypothetical

system, while an arterial duct connects each
arch to its companion pulmonary artery
(Figure 9.38). Persistent patency of the
entire double arch obviously will cause
problems (Figures 9.39, 9.40), while
inappropriate interruption or atresia at any
part of the ring can produce multiple
variations of abnormal anatomy24. Such
situations are illustrated in Figures 9.41
and 9.42, which show a left aortic arch with
an aberrant right subclavian artery. Stewart
342
Tracheo-oesophageal
pedicle
Right-sided arch
Left-sided arch
RSCA
RCCA
LCCA
LSCA
Post.
Fig. 9.39 In this heart, there is persistence of both the right- and
Right
Left-sided
arterial
ligament
Left
Ant.
left-sided aortic arches, with the origin of the common carotid (RCCA,
LCCA) and subclavian (RSCA, LSCA) arteries in symmetrical fashion
from the superior surface of the arches. There is a ligamentous
left-sided arterial duct. The arrangement produces obvious
compression of the tracheo-oesophageal pedicle.
Tracheo-oesophageal
pedicle
RCCA
LCCA
Ant.
RSCA
Left
Right
LSCA
Post.
and colleagues23 provided an exhaustive list

of the potential malformations, which they
catalogued in complex alphanumeric
fashion. Our preference is for descriptive
Fig. 9.40 The reconstructed dataset shows persistence of both the

right- and left-sided aortic arches, again with the origin of the
common carotid (RCCA, LCCA) and subclavian (RSCA, LSCA) arteries
in symmetrical fashion from the superior surface of the arches.
analysis rather than alphanumeric

notation24. It should be noted that, as in the
latter example of focal atresia, the atretic
segment may not persist as a brous cord,
and may not necessarily cause compression

of the trachea or oesophagus. In addition,
the size of the component parts of the ring
is at least as important in producing
343
Diverticulum
of Kommerell
RSCA
Fig. 9.41 This cartoon shows how an aberrant origin of the right
subclavian artery (RSCA) is explained on the basis of the
hypothetical double arch. The proximal portion of the right arch is
known as the diverticulum of Kommerell, and the initial segment
of the right arch is interrupted between the right common
carotid and right subclavian arteries (star). The duct, shown in the
left-sided position (black curved line), has become ligamentous in
this example.
Ant.
Left
RCCA
Right
Post.
Trachea
LCCA
Fig. 9.42 The computed tomographic dataset shows the origin
LSCA
RSCA
symptoms as the particular anatomical

arrangement. This accounts for the high
incidence of symptoms associated with a
double aortic arch. When only a brous
cord is found, compression is not always
apparent. Figures 9.43 and 9.44 show a
duct taking origin from the left subclavian
artery, which arises from a right arch. The
of a retro-oesophageal right subclavian artery (RSCA) from a

left-sided aortic arch. The trachea, but not the oesophagus, is seen
in the reconstruction. The potentially double arch is divided
between the right subclavian and right common carotid (RCCA)
arteries. The left common carotid (LCCA) and left subclavian (LSCA)
arteries arise from the left-sided aortic arch. The arterial duct is not
seen in this reconstruction, but was left-sided. Therefore, this
patient does not have a complete vascular ring.
considerable tracheo-oesophageal
compression is not obvious until the duct is
divided, and its ends are allowed to spring
apart (Figure 9.45).
A right-sided aortic arch can exist aside
from its association with vascular rings.
The arch is right-sided when it crosses over
the main stem of the right-sided bronchus.
It may then connect to the descending aorta

on either the right or left side of the
vertebral column. Such a right-sided aortic
arch is considered abnormal with the usual
atrial arrangement, but is normal in
patients with the mirror-imaged
arrangement. In either case, it may or may
not produce clinical problems. Its chief
344
Ant.
Right
Left
Post.
Aorta
Trachea
Pulmonary
trunk
LCCA
Fig. 9.43 This computed tomographic dataset is the mirror-image
Diverticulum
of Kommerell
Arterial duct
Aberrant LSCA
of the arrangement shown in Figure 9.42, with a retro-oesophageal

origin of the left subclavian artery (LSCA) from a right-sided aortic
arch, with the dorsal part of the right arch persisting as the
diverticulum of Kommerell. In this instance, however, the left
subclavian artery continues as the left-sided arterial duct, creating a
vascular ring. The trachea, but not the oesophagus, is seen in the
reconstruction. The potentially double arch is divided between the
left subclavian and left common carotid (LCCA) arteries.
Vagus nerve
Double ligation of duct
Aberrant LSCA
Fig. 9.44 This operative view, taken through a left thoracotomy,
Ant.
Inf.
Sup.
Post.
surgical signicance lies in its association

with about one-quarter of cases of tetralogy
of Fallot, and approximately half of all
patients with common arterial trunks.
shows, as already seen in Figure 9.43, an arterial duct that arises

from an aberrant left subclavian artery (LSCA), itself coursing in a
retro-oesophageal position from a right aortic arch. The
arrangement produces a vascular ring, as shown in Figure 9.43. The
duct has been doubly ligated prior to its division. Note the size and
location of the vagus nerve and its recurrent laryngeal branch.
Patients with a right aortic arch usually

have a mirror-imaged arrangement of the
brachiocephalic arteries. This fact can be
signicant when contemplating
construction of a subclavian-to-pulmonary
arterial shunt. With mirror-imaged
branching, the rst branch of the aorta
beyond the coronary arteries is the
345
Retracted ends of divided duct
Oesophagus
Ant.
Inf.
Sup.
Fig. 9.45 After division of the duct in the patient shown in

Figure 9.44, the ends of the arch have sprung apart to free the
oesophagus. The appearance subsequent to the surgical procedure
shows the initial effect of the vascular ring.
Post.
brachiocephalic trunk, which subsequently

gives rise to the left subclavian and
common carotid arteries. Under such
circumstances, the surgeon may elect to
perform a left-sided anastomosis so as to
avoid kinking of the right subclavian artery
as it is turned down into the mediastinum.
The duct, however, usually remains a
left-sided structure, connecting the
brachiocephalic trunk to the left pulmonary
artery. When arising from a retrooesophageal left subclavian artery, the
presence of the left-sided duct will produce
a vascular ring (Figures 9.42, 9.43, 9.46)24.
The hypothetical double arch, as
proposed by Stewart and his colleagues23,
also accounts for isolation of the
brachiocephalic arteries. In these
anomalies, which can involve either the
brachiocephalic artery itself, or the
common carotid or subclavian arteries, and
which can be associated with either the
usual left-sided aortic arch or a right aortic
arch, the isolated artery arises from a
pulmonary artery via a persistently patent
arterial duct. In the example shown in
Figure 9.47, there is persistence of both the
right-sided and left-sided arterial ducts.
The aortic arch is interrupted between the
left common carotid and left subclavian

arteries, with the isolated right subclavian
artery arising via a right-sided arterial duct
from the right pulmonary artery.
PULMONARY ARTERIAL
ANOMALIES
By far the most common malformation of
the pulmonary arteries, excluding atresia or
stenosis, is for either the right or left
pulmonary artery to have an aortic origin.
Most frequently the anomalous artery arises
via the arterial duct. Although seen in the
presence of pulmonary atresia, usually
with the other lung supplied by major
systemic-to-pulmonary collateral arteries
(Figure 9.48), it can be found with the other
pulmonary artery connected to the patent
pulmonary trunk. In this arrangement, the
duct will often close with time. There will
then be apparent unilateral absence of that
pulmonary artery that was initially fed
through the duct. This is common in
tetralogy of Fallot or a double-outlet right
ventricle, but about half of the patients thus
aficted have otherwise normal hearts. In
our experience, it is rare to nd true absence
of the hilar pulmonary artery.
Less frequently, the anomalous

pulmonary artery, usually the right, can
arise directly from the ascending aorta
(Figure 9.49). This is sometimes termed a
hemitruncus, but we do not recommend
use of this term, because almost always there
are two normally formed arterial valves.
Rarely, the right pulmonary artery may take
an unusually high origin from the right side
of the ascending portion of a common arterial
trunk. Even in this setting, the descriptive
title of common trunk with anomalous origin
of the right pulmonary artery is preferable to
hemitruncus.
When the anomalously connected
pulmonary artery arises directly from the
aorta, surgical repair consists simply of
detachment from the aorta and
reattachment to the pulmonary trunk. This
is somewhat more difcult in the presence
of a common trunk, but the same basic
principle is followed for repair. When the
anomalous pulmonary artery is fed through
a duct, or connected by a ligament, it will
arise from the aortic arch or a
brachiocephalic artery. Care must be taken
during surgical reconstruction to ensure
that ductal tissue is not incorporated with
the anastomosis, as it may subsequently
346
Trachea
RSCA
Oesophagus
LSCA
Right arch
RCCA
Arterial
duct
Fig. 9.46 The specimen shows a potential double aortic arch
LCCA
that is interrupted between the left common carotid artery (LCCA)

and the left subclavian artery (LSCA), the latter arising in a
retro-oesophageal fashion from the right-sided aortic arch. Despite
the incomplete nature of the double arch, the presence of the
arterial duct, running from the left subclavian artery to the left
pulmonary artery, produces a vascular ring around the tracheooesophageal pedicle. The right common carotid (RCCA) and right
subclavian (RSCA) arteries arise from the right aortic arch.
Sup.
Right
Left
Pulmonary trunk
Inf.
RSCA
Right pulmonary
artery
LSCA
RCCA
LCCA
Pulmonary
trunk
Fig. 9.47 In this heart, there is interruption of the aortic arch

Sup.
Left
Right
Ascending
aorta
Inf.
constrict and produce stenosis at the site of

repair. In cases with unilateral absence of a
pulmonary artery, it is usual to nd
pulmonary hypertension and pulmonary
hypertensive vascular disease in the
normally connected lung, probably because
between the left common carotid (LCCA) and left subclavian (LSCA)
arteries. The ascending aorta, however, gives rise to the two
common carotid arteries only, the right subclavian artery (RSCA)
being isolated, and arising from the right pulmonary artery via
the right-sided arterial duct (white star). The descending aorta
and the left subclavian artery are fed by the left-sided arterial duct
(red star).
of its being disproportionately small and

having to receive the entire right
ventricular output25.
A much more rare anomaly is when
the left pulmonary artery arises from the
right pulmonary artery. The abnormal
left pulmonary artery originates within

the right chest (Figures 9.50, 9.51),
and passes between the trachea and
oesophagus to the left pulmonary
hilum, creating the vascular sling
(Figs. 9.52)26. This may result in repeated
347
Sup.
Arterial ligament
Left
Right
Inf.
Fig. 9.48 In this specimen, photographed from behind, the left

pulmonary artery was initially fed from a left-sided duct, which has
become ligamentous. The right lung is fed through systemic-topulmonary collateral arteries.
Collateral arteries to right lung
Pulmonary trunk
Aorta
Left
Fig. 9.49 In this operative view, taken through a median

Sup.
Inf.
Right
respiratory problems, requiring division

and transplantation of the offending
artery. As discussed earlier, the right
pulmonary artery may arise anomalously
sternotomy, the right pulmonary artery is seen arising directly from

the aorta. This should not be described as hemitruncus, as there are
separate aortic and pulmonary pathways, the pulmonary trunk
continuing as the left pulmonary artery.
from the aorta or brachiocephalic

artery, but an abnormal right pulmonary
vessel arising from the left pulmonary
artery has not, to our knowledge,
been reported. The reason for this is

not clear.
In the rare occurrence of agenesis of
the right lung, displacement of the heart
348
Descending aorta
Trachea
Arterial duct
Right pulmonary
artery
Sup.
Right
Left
Fig. 9.50 This specimen is photographed from behind. The left

Inf.
pulmonary artery arises from the right pulmonary artery. It then

extends between the trachea and the oesophagus, which has been
removed, producing the pulmonary arterial sling (see Figure 9.52).
Right pulmonary
artery
Aorta
Post.
Right
Left
Trachea
Ant.
into the right chest may create a ring-like

disposition that can obstruct the left
bronchus. The displaced heart, pulling
on the left pulmonary artery, draws the
ligament and descending aorta across
the left bronchus, compressing the
Fig. 9.51 This computed tomographic dataset shows the origin of

the left pulmonary artery from the right pulmonary artery. It
extends between the trachea and the oesophagus, producing the
pulmonary arterial sling (see Figure 9.52).
bronchus against the spinal column.

Division of the ligament, and grafting
of the aorta, may be necessary to allow
the pulmonary trunk and aorta to
spring apart and unroof the constricting
circle2729.
PERSISTENCE OF THE ARTERIAL

DUCT AND AORTOPULMONARY
WINDOW
Persistence of the arterial duct
(Figure 9.53) occupies a special place in
Right
pulmonary
artery
Oe
Left
pulmonary
artery
Fig. 9.52 The cartoon shows the arrangement known as the

pulmonary arterial sling, illustrated in Figures 9.50 and 9.51. Oe,
oesophagus; T, trachea.
Sup.
Ant.
Post.
Inf.
Aorta
Pulmonary
trunk
Fig. 9.53 The reconstructed computed tomographic

the study of cardiovascular disease, as it

was the rst congenital malformation to
be cured by operative intervention30.
Although now the duct can be closed
angiogram shows a persistently patent arterial duct (white

double-headed arrow).
safely by techniques of interventional

catheterisation31,32, its interruption
remains a paradigm of the best of
surgical science. The anatomy is almost
always predictable, and the operative

results uniformly excellent.
Because the arterial system develops
with bilateral symmetry, a persistent duct
349
350
Patent arterial duct
Pulmonary trunk
Left
Sup.
Aorta
Inf.

shows the origin of a persistently patent arterial duct from the
distal extent of the pulmonary trunk.
Right
may be either right- or left-sided, although

the latter is overwhelmingly more
common. Because the duct can persist on
either side, or bilaterally, then as discussed
earlier, it may be important as part of a
vascular ring, or in providing the origin of
an isolated brachiocephalic artery
(Figure 9.47). Persistent patency may also
play an important physiological role when
it accompanies other complex congenital
cardiovascular anomalies, such as
interruption of the aortic arch, aortic or
pulmonary valvar atresia, or discordant
ventriculoarterial connections. In this
section, we conne our discussion to the
primary congenital condition of an isolated
left-sided persistently patent arterial duct.
Although it is possible to approach the
patent duct anteriorly, and sometimes
necessary to use this route (Figure 9.54),
the normal operative approach is through a
left lateral thoracotomy (Figure 9.55). The
duct arises from the posterosuperior aspect
of the junction of the pulmonary trunk and
left pulmonary artery. It courses
posteriorly and slightly leftwards to join the
junction of the aortic arch and descending
aorta just distal to and opposite the origin of
the left subclavian artery. Its pulmonary
end is covered by a fold of pericardium, and
its aortic end by parietal pleura. It may be
confused with the aortic isthmus,

particularly in the infant, and it is possible
to identify the left pulmonary artery as a
patent duct. Even under the best
conditions, these other structures may be
mistakenly ligated in place of the duct. The
caveats of this procedure have been
elegantly reviewed33. Approached laterally,
the best anatomical guide to the duct is the
vagus nerve and its recurrent laryngeal
branch. The vagus nerve passes along the
subclavian artery, crossing over the aortic
arch before heading in a posterior direction
to disappear behind the hilum. Just at the
level of the duct, it gives off the recurrent
nerve (Figure 9.55), which then curves
beneath the inferomedial wall of the duct
before ascending along the posteromedial
aspect of the aorta into the groove between
the trachea and oesophagus.
Access to the duct may be achieved by
incising the mediastinal parietal pleura,
either between the phrenic and vagus
nerves, or more posteriorly over the aorta
itself. With either approach, the recurrent
nerve must be visualised to guard against
direct trauma or injury by traction. The
fold of pericardium extending over the
pulmonary end of the duct may be lifted
away by sharp dissection. The
posteromedial wall of the duct is rmly
attached by another more brous fold to

the bronchus. It is this rm brous fold that
prevents easy circumscription of the duct
with a right-angled clamp. To minimise the
risk of tearing the wall of the duct, this
tissue must be divided by sharp dissection.
This can be done through a superior
approach over the aortic end of the duct, or
by freeing the aorta and retracting it
medially. In the latter instance, small but
potentially troublesome bronchial vessels
may be encountered, arising from the
posterior wall of the aorta. Another
anatomical note of caution involves the
thoracic duct and its tributaries in the area
of the origin of the subclavian artery
(Figure 9.29). Division of any of these
major lymph vessels is liable to lead to
chylothorax and its attendant difculties.
Should the lymphatic trunks be
inadvertently divided, they must be ligated
to avoid chylothorax. The duct in an infant
or small child can measure from one to 15
millimetres in length and diameter. Rarely,
it can be aneurysmally dilated
(Figure 9.56)34. These may be aneurysms
of a truly patent duct, or may simply be a
dilated ductal diverticulum with a closed
pulmonary end. In general, the short and
fat duct should be cross-clamped, divided,
and oversewn to minimise the chances of
Ant.
Inf.
351
Pericardial reflection
Sup.
Post.
Vagus nerve
Fig. 9.55 In this operative view, a persistently patent arterial duct

Thoracic aorta
Patent arterial duct
is seen through a left thoracotomy (compare with Figure 9.54).

Note the location of the vagus nerve and its recurrent laryngeal
branch.
Aneurysmal duct
Ascending aorta
Descending aorta
Sup.
Pulmonary trunk
Right
Left
Fig. 9.56 The heart is photographed in anatomical orientation

Inf.
incomplete ligation or tearing of the vessel

wall. For the longer thin duct, a triple
ligation technique has proved to be safe and
effective35.
from the front. The arterial duct is patent, elongated, and

aneurysmal.
The clinical presentation of an

aortopulmonary window is often similar to
that of a common arterial trunk. The
anatomical distinction lies in the mode of
ventriculoarterial connection. Where, in a

common trunk, there is a solitary arterial
valve, an aortopulmonary window is always
associated with separate aortic and
352
Aortopulmonary
window
Descending
aorta
Aortic
valve
Sup.
Right
Left
Fig. 9.57 This specimen, shown in anatomical orientation, the
Inf.
Pulmonary trunk
aorta having been opened, shows an aortopulmonary window.

Note the presence of the separate arterial trunks and valves
proximal to the defect (see also Figure 9.46).
Duct
Left
pulmonary
artery
Aorta
Fig. 9.58 This specimen has an aortopulmonary window in the
Sup.
Ant.
Post.
Inf.
Pulmonary valve
pulmonary valves (Figure 9.57). Although

the ventricular septum is usually intact, this
defect frequently is associated with
additional congenital cardiovascular
anomalies36.
setting of the origin of the right pulmonary artery from the aorta
and interruption of the aortic arch. The heart is sectioned to
replicate the left anterior oblique subcostal echocardiographic
section. In this heart, the window (open double-headed arrow) has
some length. Note also the separate walls of the arterial trunks
proximal to the window (closed arrow).
The defect itself is located usually in the

right lateral wall of the pulmonary trunk
anterior and opposite to the origin of the
right pulmonary artery, which frequently
overrides the window, or arises directly
from the aorta (Figure 9.58). This means

that its opening into the left side of the
ascending aorta is just distal to the
sinutubular junction. The window can also
be associated with the origin of one of the
coronary arteries from the pulmonary

trunk. The defect itself can take the form of
a well-demarcated short tubular channel, or
present as an extensive fenestration.
Surgical repair is best accomplished
through an incision in the ascending aorta
using standard cardiopulmonary bypass
techniques. As a general rule, closed
methods are not advisable, not even for the
more tubular defects. When the window
supplies a vital part of the circulation, it
cannot be closed without providing an
alternate source of blood for the dependent
segment of the circulation.
ANOMALIES OF THE CORONARY

ARTERIES
Congenital malformations of the coronary
arteries in otherwise normally structured
hearts have, traditionally, been categorised
as major or minor. This approach was
rooted in the belief that the so-called major
anomalies were those that produced
symptomatology, whereas the minor
lesions were thought to be of no clinical
relevance37. The potential danger of such a
classication became evident when it was
realised that some lesions categorised as
minor within this concept could be a cause
of sudden cardiac death38. Because of this
discrepancy between presumed anatomical

and revealed clinical signicance, it now
seems preferable to account for these
malformations on a descriptive basis.
Within such a descriptive categorisation,
the anomalies can be grouped into those
with an anomalous origin of the coronary
arteries from the arterial roots, those with
an anomalous course of the epicardial
coronary arteries, those with anomalous
communications between the coronary
arteries and other structures within the
heart, or combinations of such ndings.
Anomalous origin of the

coronary arteries
The coronary arteries can arise anomalously
either from the pulmonary trunk or, rarely,
from the right or left pulmonary artery.
They can also take an anomalous origin
from the aorta itself. It is the anomalous
origin from the pulmonary trunk that is of
most clinical signicance. Often described
as the BlandWhiteGarland syndrome,
the anomalous origin of the left coronary
artery from the pulmonary trunk
(Figures 9.599.61) usually presents in
infancy with ischaemia of the left ventricle
(Figure 9.62). If there is a well-developed
collateral circulation, presentation can be
353
markedly delayed, some cases not

presenting until childhood or even
adolescence. The clinical problems are
produced by the extent of ischaemia of the
left ventricular myocardium, which can be
extreme. It is important to reattach the
artery to the aorta as soon as the diagnosis is
made. In most instances, the artery arises
from the left sinus of the pulmonary trunk,
to the right-hand side of the observer
standing, guratively speaking, in the
non-adjacent sinus39. It is relatively easy to
transfer the arterial origin, together with a
button of pulmonary sinus, back to the
left-hand adjacent sinus of the aorta. It is
rare now to create an aortopulmonary
tunnel in order to reconnect the artery to the
aorta40. Either of these procedures,
nonetheless, is usually preferable to simple
ligation of the coronary artery. The pattern
that may create problems in transfer is when
the artery arises from a branch of the
pulmonary trunk rather than from the trunk
itself. Other patterns, such as the anomalous
origin of the right coronary artery from
the pulmonary trunk, do not produce the
same degree of symptomatology, and often
do not come to the attention of the surgeon.
If encountered, an anomalous right
coronary artery can be transferred back to
the aortic root readily.
Pulmonary trunk
Ant.
Sup.
Inf.
Post.
Fig. 9.59 This view through a left lateral thoracotomy shows a silk
ligature encircling a left coronary artery that is arising from the
pulmonary trunk.
354
Sup.
Post.
Ant.
Pulmonary trunk
Inf.
Circumflex artery
orientation, the main stem of the left coronary artery takes origin
from the pulmonary trunk, arising from one of the sinuses adjacent
to the aorta. It then divides into the circumex and anterior
interventricular arteries.
Circumflex artery
Aorta
Pulmonary
trunk
Post.
Left
Right
Ant.

We have already discussed the

signicance of an anomalous origin of one
coronary artery from the aorta itself in
Chapter 4. As we showed, the coronary
shows an anomalous origin of the left coronary artery from the

pulmonary trunk. Note the tortuous collateral circulation (arrow)
from the right coronary artery.
arteries usually arise from the two aortic

sinuses that are adjacent to the pulmonary
trunk. In the normal heart, the left
coronary artery arises from the left-hand
facing sinus, while the right coronary artery

arises from the right-hand facing sinus.
Accessory orices supplying either the
infundibular artery, the artery to the sinus
Dilated left ventricle
355
Sup.
Ant.
Post.
Inf.
Fig. 9.62 In this anatomical specimen, photographed in

Ischaemic myocardium
node, or an artery supplying the walls of the

arterial trunks, are by no means
uncommon. These accessory branches
should not be interpreted as representing
anomalies. The circumex and anterior
descending branches of the left coronary
artery can also rarely take separate origins
from the left-hand facing sinus in an
otherwise normal heart. Usually the
arteries arise within the coronary aortic
sinuses, but origin at the sinutubular
junction, or just above it, should not be
considered abnormal. High origin,
however, or an oblique course of a coronary
artery through the wall, particularly when
crossing the peripheral end of a zone of
apposition between the valvar leaets,
should be considered anomalous, as should
the origin of either coronary artery from an
inappropriate sinus or, as occurs very
rarely, from the non-facing aortic sinus41.
The origin from an inappropriate sinus is of
greatest importance, as this is now well
established to be a harbinger of sudden
cardiac death42. As we showed in
Chapter 4, either coronary artery can arise
from an inappropriate sinus. The artery
then courses between the arterial trunks,
typically crossing the peripheral end of the
zone of apposition between the leaets
anatomical orientation, the left ventricle is dilated secondary to an

anomalous origin of the left coronary artery from the pulmonary
trunk, while the ventricular myocardium is obviously ischaemic.
guarding the two aortic coronary sinuses.

This is described as an intramural
arrangement (Figure 9.63). Surgical
unroong is not difcult, but whether such
relief is always necessary has still to be
determined.
The nding of a single coronary artery
also represents an anomalous origin from
the aortic root. This can be found in two
patterns. In the rst, all three coronary
arteries arise from the same aortic sinus,
with one, two, or three orices within the
sinus. Usually it is the right-hand sinus
that gives origin to the coronary arteries.
There is then an associated anomalous
epicardial course of the branches of the left
coronary artery as they reach the
anticipated locations. As discussed in
Chapter 4, this can involve the anterior
interventricular branch tracking between
the aorta and the pulmonary trunk, with
the potential for its constriction.
Alternatively, the main stem of the left
coronary artery can run across the
subpulmonary infundibulum, tracking
towards the left atrial appendage before
dividing into the circumex and anterior
interventricular arteries (Figure 9.64). The
solitary coronary artery can also arise from
the left aortic sinus, and the right coronary
artery can then course between the aortic

root and the subpulmonary infundibulum
to reach the right atrioventricular groove
(Figure 9.65). Yet another pattern is for
the right coronary artery to take its normal
origin from the right-hand facing sinus,
but then to continue beyond the crux as
the circumex artery, and to terminate at
the obtuse margin as the anterior
interventricular artery (see Chapter 4).
This pattern probably has no clinical
signicance. In addition to an anomalous
origin, congenital malformations can also
afict normally attached arteries,
producing the effect of an anomalous
origin. Particularly important in this
respect is congenital atresia of the main
stem of the left coronary artery
(Figure 9.66). This can be another
substrate for sudden death in an adolescent
or young adult43. Unfortunately, this
anomaly is unlikely to be diagnosed prior
to the event, when it could readily be
treated by surgical manoeuvres.
Anomalous epicardial course

of the coronary arteries
Although an anomalous epicardial course
can be found in otherwise normally
356
Sup.
Fig. 9.63 The tubular aorta has been removed from the aortic
Left
Right
Right aortic sinus
Inf.
root, showing the origin of the right coronary artery from the left
coronary aortic sinus, with intramural coursing across the
peripheral attachment of the valvar leaets at the sinutubular
junction (arrow).
Left main stem
Solitary coronary artery

Left
Inf.
Sup.
Right
structured hearts, it is seen more

frequently in hearts that are themselves
malformed, such as those with discordant
ventriculoarterial connections or a
common arterial trunk. The clinical
signicance of these anomalies has yet to
be fully determined. Some are found as
sternotomy, shows a heart in which all the coronary arteries arise

from a solitary coronary artery that takes origin from the righthand adjacent aortic sinus. The main stem, which divides beneath
the left appendage to become the circumex and anterior
interventricular arteries, crosses the origin of the pulmonary trunk
from the right ventricle.
chance observations at autopsy, such as

the origin of the circumex coronary
artery from the right coronary artery, with
passage through the transverse sinus to
reach the left atrioventricular groove
(Figure 9.67). Others may be of
signicance for sudden death, such as the
passage between the aorta and the

pulmonary trunk as discussed in
Chapter 4. In this respect, muscular
bridging of the epicardial coronary
arteries (Figure 9.68) may also be of
relevance, although the signicance of this
nding has still to be established.
357
Infundibulum
Aorta
Fig. 9.65 This computed tomographic dataset shows the origin of
Ant.
Left
Circumflex artery
Right
Post.
the right coronary artery from the anterior interventricular artery,

with subsequent coursing between the subpulmonary
infundibulum and the aortic root. There was a solitary coronary
artery arising from the left coronary aortic sinus. Note the
narrowed prole of the right coronary artery.
Atretic left coronary artery
Sup.
Right
Fig. 9.66 This view of the opened aortic root,

Left
Inf.
Enlarged right coronary artery
Anomalous communications
of the coronary arteries
Fistulous communications between the
coronary arteries and the ventricles are seen
most frequently when atresia of an outow
tract is seen in the setting of an intact
ventricular septum, notably in pulmonary

atresia with an intact ventricular septum
(see Chapter 7, page 235 and Figure 7.157).
Such communications can also be found
in otherwise normally structured hearts,
and the anomalous artery can be joined to
any other cardiac structure. The right
photographed in anatomical orientation,

shows atresia of the main stem of the left
coronary artery. Note the enlarged orice of
the right coronary artery. The specimen came
from an adult who died suddenly.
coronary artery is most frequently

involved, and it may connect to the right
atrium (Figures 9.69, 9.70), the superior
caval vein, the coronary sinus, the right
ventricle, the pulmonary trunk, a
pulmonary vein, or the left ventricle.
The stulas can also take origin from the
358
Retro-aortic
circumflex
artery
Aorta
Right
coronary
artery
Sup.
Post.
Artery to
sinus node
Ant.
Fig. 9.67 This infant heart is photographed in anatomical

orientation. The circumex coronary artery arises from the right
coronary artery, and passes through the transverse sinus, behind
the aorta, to reach the left atrioventricular groove.
Inf.
Myocardial
bridging
Sup.
Right
Left
Inf.
Fig. 9.68 In this heart, photographed from the front in

anatomical orientation, there is extensive myocardial bridging
across the anterior interventricular artery.
Normal right coronary artery
359
Left
Sup.
Inf.
Right
Clamp on appendage
Fistula
sternotomy, with the right atrial appendage retracted, shows a

stula running from the right coronary artery to the base of the
appendage. The white loop is placed around the normal distal
segment of the right coronary artery, while the red loop encircles
the stula itself.
Normal coronary artery
Left
Inf.
Sup.
Right
Ends of divided fistula
Fig. 9.70 The broad stula shown in Figure 9.69 has been divided.
left coronary artery (Figure 9.71). The

communication itself can be a large solitary
orice, or a complex worm-like aneurysmal
cavity. When the communication is simple
and large, shunting across it can be
considerable and the artery itself can be
markedly dilated. Steals can also result,
with consequent ischaemia in the area of
myocardium from which the steal has

occurred.
Aortoventricular tunnels
These tunnels are rare lesions. The
anatomical arrangement is difcult to
understand, as the essence of the lesions
is to bypass the hinge of an aortic valvar

leaet (Figure 9.72). Typically, the
tunnels extend between the aorta and
the left ventricle, although aorto-right
ventricular tunnels do exist
(Figure 9.73). It is the ones
communicating with the left ventricle
that are most frequent44. The anomalous
360
Post.
Right
Left
Ant.
Aorta
Fistula
Termination in
left ventricle
shows a stula taking origin from the left coronary artery (arrow)
and terminating in the left ventricle.
Sup.
Right coronary leaflet
Post.
Ant.
Inf.
Aorta
Fig. 9.72 The aortic root has been opened and photographed
Left ventricle
channel, coursing from the

left ventricle between the base of the
aficted aortic sinus and the leaet it
from the front. The red cord is passing through an aorto-left

ventricular tunnel, which bypasses the hinge of the right coronary
aortic valvar leaet.
supports, extends through the tissue

plane that separates the aortic and
pulmonary roots. The base of the tunnel
is supported by the ventricular

myocardium, while its roof is made up of
arterial wall.
High origin of both coronary arteries
361
Aortoventricular tunnel
Sup.
Right
Left
Inf.
Fig. 9.73 The aortic root has been opened and is photographed
Right aortic leaflet
References
1. Anderson RC, Heilig W, Novick R, Jarvis
C. Anomalous inferior vena cava with
azygos drainage: so-called absence of the
inferior vena cava. Am Heart J 1955; 49:
318322.
2. Moller JH, Nakib A, Anderson RC,
Edwards JE. Congenital cardiac disease
associated with polysplenia: a
developmental complex of bilateral leftsidedness. Circulation 1967; 36: 789799.
3. Venables AW. Isolated drainage of the
inferior vena cava to the left atrium. Br
Heart J 1963; 25: 545548.
4. Winter FS. Persistent left superior vena
cava: survey of world literature and report
of 30 additional cases. Angiology 1954; 5:
90132.
5. Bankl H. Congenital Malformations of the
Heart and Great Vessels. Baltimore, MD:
Urban and Schwarzenberg, 1977; p 194.
6. Agnoletti G, Annechino F, Preda L, Borghi
A. Persistence of the left superior caval
vein: can it potentiate obstructive lesions of
the left ventricle? Cardiol Young 1999; 9:
285290.
7. Edwards JE, DuShane JW. Thoracic
venous anomalies. I. Vascular connection
between the left atrium and the left
innominate vein (levoatriocardinal vein)
associated with mitral atresia and premature
closure of the foramen ovale (case 1). II.
Pulmonary veins draining wholly to the
8.
9.
10.
11.
12.
13.
from behind. The aortic end of a tunnel is seen, which courses

through the supraventricular crest and opens into the right
ventricular infundibulum.
ductus arteriosus (case 2). Arch Pathol

1950; 49: 517537.
Pinto CAM, Ho SY, Redington A,
Shinebourne EA, Anderson RH.
Morphological features of the
levoatriocardinal (or pulmonary-tosystemic collateral) vein. Pediatr Pathol
1993; 13: 751761.
Lucas R Jr, Woolfrey BF, Anderson RC,
Lester RG, Edwards JE. Atresia of the
common pulmonary vein. Pediatrics 1962;
29: 729739.
DeLisle G, Ando M, Calder AL, et al.
Total anomalous pulmonary venous
connection: report of 93 autopsied
cases with emphasis on diagnostic and
surgical considerations. Am Heart J 1976;
91: 99122.
Gupta SK, Gulati GS, Juneja R,
Devagourou V. Total anomalous
pulmonary venous connection with
descending vertical vein: unusual drainage
to azygos vein. Ann Pediatr Cardiol 2012;
5: 188190.
Neill CA, Ferencz C, Sabiston DC,
Sheldon H. The familial occurrence of
hypoplastic right lung with systemic arterial
supply and venous drainagescimitar
syndrome. Bull Johns Hopkins Hosp 1960;
107: 121.
Chauvin M, Shah DC, Haisseguerre M,
Marcellin L, Brechenmacher, C. The
anatomic basis of connections between the
coronary sinus musculature and the left
14.
15.
16.
17.
18.
19.
20.
atrium in humans. Circulation 2000; 101:

647652.
Brom AG. Narrowing of the aortic isthmus
and enlargement of the mind. J Thorac
Ho SY, Anderson RH. Coarctation, tubular
hypoplasia and the ductus arteriosus: a
histological study of 35 specimens. Br Heart
J 1979; 41: 268274.
Elzenga NJ, Gittenberger-de-Groot AC.
Localised coarctation of the aorta. An age
dependent spectrum. Br Heart J 1983;
49: 317323.
Becker AE, Becker MJ, Edwards JE.
Anomalies associated with coarctation of
the aorta. Particular reference to infancy.
Circulation 1970; 41: 10671075.
Hamilton WF, Abbott ME. Coarctation of
the aorta of the adult type: Part I. Complete
obliteration of the descending arch at
insertion of the ductus in a boy of fourteen;
bicuspid aortic valve; impending rupture of
the aorta; cerebral death. Part II. A
statistical study and historical retrospect of
200 recorded cases, with autopsy, of
stenosis or obliteration of the descending
arch in subjects above the age of two years.
Am Heart J 1928; 3: 381421.
Lerberg DB. Abbotts artery. Ann Thorac
Surg 1982; 33: 415416.
Pierpont M E M, Zollikofer CL, Moller JH,
Edwards JE. Interruption of the aortic arch
with right descending aorta. Pediatr Cardiol
1982; 2: 153159.
362
21. Ho SY, Wilcox BR, Anderson RH,

Lincoln JCR. Interrupted aortic
archanatomical features of surgical
signicance. Thorac Cardiovasc Surg 1983;
31: 199205.
22. Barry A. Aortic arch derivatives in the
human adult. Anat Rec 1951; 111: 221238.
23. Stewart JR, Kincaid OW, Edwards JE.
An Atlas of Vascular Rings and Related
Malformations of the Aortic Arch System.
Springeld, IL: Charles C. Thomas, 1964.
24. Ramos-Duran L, Nance JW, Schoepf UJ,
et al. Developmental aortic arch anomalies
in infants and children assessed with CT
angiography. AJR Am J Roentgenol 2012;
198: W466W474.
25. Pool P E, Vogel JHK, Blount SG Jr.
Congenital unilateral absence of a
pulmonary artery. The importance of ow
in pulmonary hypertension. Am J Cardiol
1962; 10: 706732.
26. Contro S, Miller RA, White H, Potts WJ.
Bronchial obstruction due to pulmonary
artery anomalies. I. Vascular sling.
Circulation 1958; 17: 418423.
27. Maier HC, Gould WJ. Agenesis of the lung
with vascular compression of the
tracheobronchial tree. J Pediatr 1953; 43:
3842.
28. Harrison MR, Hendren WH. Agenesis of
the lung complicated by vascular
compression and bronchomalacia. J Pediatr
Surg 1975; 10: 813817.
29. Harrison MR, Heldt GP, Brasch RC, de
Lorimier AA, Gregory GA. Resection of
distal tracheal stenosis in a baby with
30.
31.
32.
33.
34.
35.
36.
agenesis of the lung. J Pediatr Surg 1980;

15: 938943.
Gross RE. Surgical management of patent
ductus arteriosus with summary of four
surgically treated cases. Ann Surg 1939;
110: 321356.
Ali Kahn MA, Al Yousef S, Mullins CE,
Sawyer W. Experiences with 205
procedures of transcatheter closure of
ductus arteriosus in 182 patients, with
special reference to residual shunts and long
term follow-up. J Thorac Cardiovasc Surg
1992; 104: 17211727.
Lloyd TR, Fedderly R, Mendelsohn AM,
Sandhu SK, Beekman RH. Transcatheter
occlusion of patent ductus arteriosus
with Gianturco coils. Circulation 1993;
88: 14141420.
Pontius RG, Danielson GK, Noonan JA,
Judson JP. Illusions leading to surgical
closure of the distal left pulmonary artery
instead of the ductus arteriosus. J Thorac
Mendel V, Luhmer J, Oelert H. Aneurysma
des Ductus arteriosus bei einem
Neugeborenen. Herz 1980; 5: 320323.
Wilcox BR, Peters RM. The surgery of
patent ductus arteriosus: a clinical report of
14 years experience without an operative
death. Ann Thorac Surg 1967; 3: 126131.
Faulkner SL, Oldham RR, Atwood GF,
Graham TP. Aortopulmonary
window, ventricular septal defect and
membranous pulmonary atresia with a
diagnosis of truncus arteriosus. Chest 1974;
65: 351353.
37. Ogden JA. Congenital anomalies of the

coronary arteries. Am J Cardiol 1970; 25:
474479.
38. Becker AE. Variations of the main coronary
arteries. Edited by Becker AE, Losekoot T,
Marcelletti C, Anderson RH, Edinburgh,
Churchill Livingstone. Pediatr Cardiol
1983; 3: 263277.
39. Smith A, Arnold R, Anderson RH, et al.
Anomalous origin of the left coronary artery
from the pulmonary trunk. Anatomic
ndings in relation to pathophysiology and
surgical repair. J Thorac Cardiovasc Surg
1989; 98: 1624.
40. Takeuchi S, Imamura H, Katsumoto K,
et al. New surgical method for repair of
anomalous left coronary artery from the
pulmonary artery. J Thorac Cardiovasc Surg
1979; 78: 711.
41. Ishikawa T, Otsuka T, Suzuki T. Anomalous
origin of the left main coronary artery from
the non-coronary sinus of Valsalva. (Letter)
42. Kragel AH, Roberts WC. Anomalous origin
of either right or left main coronary artery
from the aorta with subsequent coursing
between aorta and pulmonary trunk:
analysis of 32 necropsy cases. Am J Cardiol
1988; 62: 771777.
43. Debich DE, Williams KE, Anderson RH.
Congenital atresia of the orice of the left
coronary artery and its main stem. Int J
Cardiol 1989; 22: 398404.
44. McKay R, Anderson RH, Cook AC. The
aorto-ventricular tunnels. Cardiol Young
2002; 12: 563580.
10
Positional anomalies
of the heart
364
The surgical problems posed by cardiac

malformations may be considerably
increased when the heart itself is in an
abnormal position. This is, in part, due to
the unusual anatomical perspective
presented to the surgeon because of the
malposition, and also to the abnormal
locations of the cardiac chambers, which
may necessitate approaches other than those
already discussed. Cardiac malposition
itself, nonetheless, does not constitute a
diagnosis. Any normal or abnormal
segmental combination can be found in a
heart that, itself, is abnormally located. The
heart may be normal, despite its abnormal
location, but extremely complex anomalies
are frequently present. Consequently, the
very presence of an abnormal cardiac
position emphasises the need for a full and
detailed segmental analysis of the heart. All
the rules enunciated in Chapter 6 apply
should the heart not be in its anticipated
position. In this chapter, we conne
ourselves to a description of abnormally
positioned hearts, giving a more detailed
discussion for specic types of malposition.
We conclude with a review of the surgical
signicance of isomerism of the atrial
appendages, which is generally agreed to be
one of the major harbingers of abnormal
cardiac position. We emphasise the need to

segregate the syndromes, preferably into the
subsets of right versus left isomerism, as the
prognosis is markedly different for the two
variants.
THE ABNORMALLY POSITIONED

HEART
Account should be taken of not only an
abnormal position of the heart within the
chest, but also the direction of its apex.
These are independent features. In the
normal individual, the heart is positioned
with its apex to the left, and with twothirds of its bulk to the left of the midline.
A mirror-imaged atrial arrangement is
usually accompanied by a mirror-imaged
cardiac arrangement. The expected
arrangement is for the cardiac apex to
point to the right, with the greater part of
the cardiac mass in the right hemithorax.
In the setting of isomerism of the atrial
appendages, there is no norm. Thus, for
all patients with isomerism, and equally
for those with the usual or mirror-imaged
atrial arrangements and abnormally
positioned hearts, it is necessary to have a
system that permits a simple and straightforward description of the abnormal
Morphologically
right atrial appendage
cardiac position. In the past, formidable

conventions were constructed, using
terms such as dextrocardia,
dextroposition, dextrorotation, or arcane
variants such as pivotal dextrocardia1. In
practice, the only requirement is for a
system that accounts for the two major
features in independent fashion, namely
the position of the cardiac mass relative to
the silhouette of the chest, and the
direction of the cardiac apex. In the past,
we have dened dextrocardia as the
situation in which the heart is in the right
chest, with the apex pointing to the right
(Figure 10.1). But what happens on the
rare occasion when the heart is in the right
chest, but its apex is pointing to the left?
This situation is readily, and
unambiguously, described as the heart
located in the right chest, with its apex
pointing to the left. Those who wish to
give nominative denitions to the
arrangement are free to do so, but the
descriptive approach is entirely adequate,
and much less liable to misconstruction.
Thus, the heart can be described as being
mostly in the left chest, mostly in the right
chest, or symmetrical. The apical
orientation can be to the left, to the right,
or to the middle.
Left
Inf.
Sup.
Right
Aorta
Apex to right
Pulmonary trunk
shows a patient with a mirror-imaged arrangement of the organs.

The heart is mostly in the right chest, with the apex pointing to the
right (arrow).
EXTERIORISATION OF THE HEART

The description given earlier does not
account for the most severe cardiac
malposition, namely exteriorisation of the
heart, or ectopia cordis. It has been said that
the heart can protrude from the thoracic
cavity in cervical, thoracic,
thoracoabdominal, or abdominal positions.
A previous review2 questioned the
existence of the cervical and abdominal
variants, arguing that all cases can be

described as thoracic or thoracoabdominal.
In the thoracic type, which is the most
common, there is a sternal defect and
absence of the parietal pericardium. The
heart protrudes directly from the thorax3.
There is usually an associated omphalocele,
and the thoracic cavity is small.
In the thoracoabdominal type, the sternal
defect is usually conuent with deciencies
of the abdominal wall and diaphragm, so the
365
heart and various abdominal organs are

displaced into an omphalocele. A
variant of this abnormality is seen in
patients with Cantrells syndrome4. These
patients have a cleft of the lower sternum.
The heart is beneath the skin in the upper
epigastrium, and is associated with an
omphalocele (Figure 10.2). When the skin
was opened in the patient shown in
Figure 10.2, the heart was seen to occupy a
position in the midline (Figure 10.3),
Collapsed umbilical hernia
Left
Fig. 10.2 This picture, taken from the right side of the
Sup.
Inf.
Right
epigastrium with the patient supine, shows the heart bulging

through the skin (star) because of a cleft in the lower sternum and
diastasis of the rectus muscles. An associated abdominal hernia is
collapsed.
Pulmonary trunk
Left
Sup.
Inf.
Right
Aorta
Fig. 10.3 The skin and upper sternum have been opened in the
patient shown in Figure 10.2, revealing the heart to be positioned

in the midline, with the apex pointing to the middle of the chest
(arrow).
366
extending towards the omphalocele

through a diaphragmatic defect.
These patients frequently have
complex intracardiac defects, often
including a diverticulum of the left
ventricle.
abnormally positioned hearts, therefore, it

is essential that the surgeon establish the
locations of the chambers within the
abnormally located organ so that the
operation can be planned appropriately.
RIGHT-SIDED HEART
CRISS-CROSS AND
SUPEROINFERIOR VENTRICLES
Our descriptive system provides no

information as to whether the abnormal
location of the heart is the result of
congenital malformation. The heart may be
in the right chest secondary to a pulmonary
defect, or because of gross enlargement of
its right-sided chambers. In either case, the
problems of surgical access are similar.
Indeed, there is no reason why, in a patient
with a lesion such as congenitally corrected
transposition, the heart should not be
positioned in the right chest because of
pulmonary problems or right-sided
hypertrophy.
There are certain lesions, nonetheless,
that spring to mind when the heart is
located in the right chest with the usual
atrial arrangement, or when the heart is
left-sided in patients with mirror-imaged
atrial chambers. The most notable of these
is, indeed, congenitally corrected
transposition. In this lesion, of course, not
all the cardiac chambers are in their
expected positions. In all patients with
With the sophistication of modern

diagnostic techniques, it is unlikely that the
surgeon will be presented with a patient
having an abnormally located heart without
a full preoperative diagnosis. Particular
arrangements of the cardiac chambers can
still give major difculties in diagnosis,
notably the arrangements known as crisscross hearts, or superoinferior ventricles.
In these anomalies, the ventricular
relationships, or more rarely the ventricular
topology, when topology is dened as
proposed in Chapter 6, are not as
anticipated for the given atrioventricular
connections5. In a patient with congenitally
corrected transposition and the criss-cross
arrangement, the morphologically right
ventricle will be predominantly right-sided
rather than in its anticipated left-sided
position6. This is a consequence of rotation
of the ventricular mass in its long axis
(Figure 10.4). The distribution of the
coronary arteries is of considerable help in
determining the position of the ventricles
Right-sided
left ventricle
Left-sided
right ventricle
Anterior
right ventricle
because, in congenitally corrected

transposition, the anterior interventricular
coronary artery arises from the right-sided
coronary artery. Thus, in a heart that at
rst sight appears to represent simple
transposition, nding the anterior
interventricular artery arising from the
right-sided coronary artery should alert the
surgeon to the possible diagnosis of
discordant atrioventricular connections
and congenitally corrected transposition.
The criss-cross arrangement, perhaps
better described as twisted atrioventricular
connections7, can also be found in the
setting of concordant atrioventricular
connections, when the morphologically
right ventricle will be left-sided
(Figures 10.5, 10.6). When the
atrioventricular connections are twisted so
as to produce the criss-crossing
arrangement, usually there is an additional
superiorinferior relationship of the
ventricles. This is a consequence of the
cardiac mass being tilted as well as rotated
(Figure 10.7), so that both criss-crossed
and superoinferior arrangements are
present. Again, the interventricular
coronary arteries are useful because they
indicate the plane of the ventricular
septum, serving as excellent guides to the
position of the ventricular cavities. The
distribution of the conduction tissue in
these bizarre hearts, however, is governed

orientation, illustrates the rotational abnormality
around the long axis (inset) that produces the socalled criss-cross abnormality. The left-hand panel
shows a heart with congenitally corrected
transposition in the absence of rotation around
the long axis. The morphologically left ventricle is
right-sided. Subsequent to rotation (right-hand
panel), the morphologically right ventricle
occupies a right-sided position, although still
connected discordantly to the morphologically left
atrium. The arrows in the right-hand panel show
the crossing atrioventricular valves, the dotted line
depicting the posterior connection to the
morphologically left ventricle, which has rotated
to a left-sided position.
367
Fontan
conduit
Morph.
left atrium
Morph. RV
Morph. LV
Post.
Right
Left
Ant.
Oversewn
pulmonary root
Fig. 10.5 The reconstructed three-dimensional magnetic

resonance image, shown in cross-section, illustrates the twisted
atrioventricular connections (arrows) that produce the criss-cross
heart. In this patient, there are concordant atrioventricular
connections, so the twisting of the ventricular mass places the
morphologically right ventricle (Morph. RV) in a left-sided position
relative to the morphologically left ventricle (Morph. LV). As shown
in Figure 10.6, both arterial trunks arose from the morphologically
right ventricle.
Aorta
Morph. RV
Morph. LV
Sup.
Post.
Ant.
Fontan conduit
Inf.
by the connections between the chambers,

and not by their position. These hearts are
often further complicated by the presence
of straddling and overriding
Fig. 10.6 The reconstructed three-dimensional magnetic

resonance image is from the patient shown in Figure 10.5, but
viewed from the right side. The morphologically right ventricle
(Morph. RV) is superiorly located relative to the morphologically
left ventricle (Morph. LV), but as shown by the black arrows, it
retains its right-hand topology despite being left-sided. Both
arterial trunks arise from the right ventricle, with the aorta in a leftsided position. The pulmonary trunk has been divided and
oversewn, and a conduit placed from the inferior caval vein to the
pulmonary arteries so as to create the Fontan circulation.
atrioventricular valves. These abnormal

relationships can be found with any
combination of atrioventricular and
ventriculoarterial connections, with a
double-outlet right ventricle and left-sided

aorta being present in the heart shown in
Figure 10.6. Even in these rare situations,
the topological arrangement of the
368
Morph. left ventricle

uppermost
Morph. right ventricle

uppermost
Usual side-by-side
ventricles
Aorta
orientation, shows the tilting along the long

axis of the ventricular mass in a heart with
congenitally corrected transposition, which
usually has side-by-side ventricles (central
panel). The tilting produces a superoinferior
relationship of the ventricles, almost always in
association with twisting of the
atrioventricular connections (see Figures 10.4
to 10.6). Depending on the direction of tilting,
either the morphologically (Morph.) left
ventricle (left-hand panel) or the
morphologically right ventricle (right-hand
panel) can be positioned uppermost. Most
usually it is the right ventricle that assumes the
superior location. As shown in Figure 10.6,
superoinferior ventricular relationships can
also be found when the atrioventricular
connections are concordant.
Sup.
Pulm.
trunk
Left
Right
Inf.
Tricuspid
valve
Fig. 10.8 In the heart shown in Figure 10.6, the right ventricle
ventricular mass is as anticipated for the

atrioventricular connections. Thus, when
there is rotation of the ventricular mass
sufcient to produce crossing of the
atrioventricular connections, this does not
disturb the intrinsic relationships of the
two ventricles7. In exceedingly rare
with a double outlet showed right-hand topology despite the

twisted atrioventricular connections. In this image, the
morphologically right ventricle exhibits left-hand topology (black
arrows), again with a double outlet from the ventricle. The
atrioventricular valve, however, is morphologically tricuspid, and
connects the right ventricle to the usually positioned right atrium.
Despite the left-hand topology, the atrioventricular connections in
this heart are concordant. This is a very rare example of segmental
disharmony5. Pulm., pulmonary.
circumstances, however, the ventricles can

not only occupy unusual positions, but the
topology is not as expected for the existing
atrioventricular connections. Hence, when
the usual atrial arrangement coexists with
concordant atrioventricular connections,
the ventricular topology is usually right-
handed (Figure 10.6). On occasion,

nonetheless, the right atrium can be joined
to a morphologically right ventricle that
shows left-hand ventricular topology, with
the right ventricle being additionally leftsided (Figure 10.8). This situation is one of
the rare occasions when it is necessary to
369
Morphologically left atrial appendage
Left
Inf.
Sup.
Right
Morphologically right atrial appendage
describe ventricular topology in addition to

accounting for the atrioventricular
connections5.
HEARTS WITH ISOMERIC ATRIAL

APPENDAGES
The problems occurring in hearts with
isomeric atrial appendages have been
mentioned already. Hearts with this
arrangement are not only found in unusual
positions, but are almost always associated
with an abnormal arrangement of the
thoracoabdominal organs; hence the
popular rubric of visceral heterotaxy. Many
will continue to classify these patients in
terms of asplenia and polysplenia8. It is
of greater value for the surgeon to base any
system of categorisation on the morphology
of the atrial appendages. Splenic
morphology does not always correspond to
the atrial anatomy, itself based on the
extent of the pectinate muscles relative to
the atrial vestibules. There is a greater
correspondence between the anatomy of
the appendages as thus determined and
what is expected of the splenic syndromes
than between these syndromes and splenic
morphology9,10. More important, it is of
little consequence to the cardiac surgeon at
the time of operation whether his or her

sternotomy, shows the different morphology of the
morphologically right as opposed to the morphologically left atrial
appendages in a patient with the usual atrial arrangement.
patient has one spleen, multiple spleens, or

no spleen at all. Determining the
morphology of the appendages brings
attention directly to the heart, enabling the
surgeon working in the operating room to
make the diagnosis of isomerism
immediately, even if this had not been
predicted by the preoperative studies.
The surgeon can readily distinguish the
appendages as being either
morphologically right or left (Figure 10.9).
The surgeon should always conrm
whether the patient possesses lateralised
atrial appendages. The nding of isomeric
left (Figure 10.10) or right (Figure 10.11)
appendages should immediately alert the
surgeon to potential problems over and
above those anticipated in the patient with
the usual or a mirror-imaged atrial
arrangement. It is now also possible to
distinguish directly the presence of
isomeric atrial appendages using computed
tomography (Figures 10.1210.15), while
use of other three-dimensional techniques
such as magnetic resonance imaging will
readily reveal the arrangement of the
bronchial tree and the abdominal organs.
There should be no problem, therefore, in
distinguishing those patients having
isomeric right as opposed to isomeric left
appendages. In hearts from patients with
isomeric right appendages (Figures 10.16

10.18), it is the rule to nd complex
intracardiac anomalies, usually with the
absence of the spleen. The pulmonary
veins will be connected in a totally
anomalous fashion even when they are
joined to the heart (Figure 10.19). Most
frequently, there are major anomalies of
systemic venous drainage. A common
atrioventricular valve is usually present
(Figures 10.16 10.18), often with a
double-inlet ventricle (Figure 10.19).
Pulmonary stenosis or atresia is frequently
found, and there are bilateral sinus
nodes1113. Increasing operative
experience now shows that even the most
complex combinations can be treated
surgically.
Although isomerism of the
morphologically right appendages is almost
always accompanied by severe intracardiac
malformations, this is not necessarily the
case when there are isomeric left
appendages (Figures 10.2010.22). Thus,
the surgeon is more likely to be confronted
with an undiagnosed case in the setting of
left isomerism. Therefore, it becomes
important to know that the sinus node is in
an anomalous position. It is usually
hypoplastic. If it can be found, it will be
located close to the atrioventricular
370
Left
Sup.
Inf.
Morphologically
left atrial
appendage
Right
Aorta

sternotomy, shows that the appendage on the right side of the
heart has left morphology. In this patient, the left-sided appendage
was also morphologically left, so the patient had isomerism of the
morphologically left atrial appendages.
Right
superior
caval
vein
Left
Fig. 10.11 In this patient, the left-sided atrial appendage is

Inf.
Sup.
Left superior
caval vein +
terminal groove
Right
broad-based, with a wide junction to the body of the atrium. Note

that the left-sided superior caval vein joins to the atrial roof, and
that there is a left-sided terminal groove. The right-sided
appendage was also of right morphology. The patient has
isomerism of the right atrial appendages.
371

Sup.

shows a patient with a right-sided morphologically (Morph.) right
atrial appendage, the right-sided atrium joining to a
morphologically right ventricle.
Ant.
Post.
Inf.
Sup.
Ant.
Post.
Inf.
Aorta
Fig. 10.13 The image shows the left side of the heart in
Figure 10.12 reconstructed. The left-sided atrial appendage also

has a broad base, and is morphologically (morph.) right. The
patient has isomerism of the morphologically right appendage.
Note the incomplete left ventricle in the posteroinferior position
(star). The aorta arises from the dominant right ventricle.
372
Ant.
Left
Right
Aorta
Post.
Fig. 10.14 The cross-sectional image is from the same dataset as

shown in Figures 10.12 and 10.13. Both appendages (stars) are
broad-based (black double-headed arrows), with pectinate muscles
extending to the crux (white arrows; see also Figure 10.18). There is
obvious isomerism of the right atrial appendages (compare with
Figure 10.15).
Sup.
Left
Right
Aorta
Inf.
Common atrium
Fig. 10.15 The computed tomogram in this patient, seen from

the front, shows the presence of two narrow appendages (stars)
with narrow necks (black double-headed arrows). This patient has
isomerism of the morphologically left atrial appendages (compare
with Figure 10.14).
Sup.
Ant.
Post.
Inf.
Septal strand
Fig. 10.16 The heart has been opened through the right
Pectinate muscles to crux
atrioventricular junction close to the crux, and the parietal wall

spread upwards. The pectinate muscles encircle the right-sided
vestibule, indicating that the right-sided appendage is
morphologically right. Note the common atrioventricular valve
(star), and absence of the coronary sinus.

Sup.
Post.
Ant.
Inf.
Septal strand
Fig. 10.17 The left side of the heart shown in Figure 10.16 has
been opened through an incision close to the crux. The pectinate
muscles also encircle the left-sided vestibule, showing that there
are morphologically right atrial appendages bilaterally. The star
shows the common atrioventricular valve.
373
374
Common atrioventricular junction

Sup.
Right
Left
Inf.
Fig. 10.18 In this heart, the atrial chambers have been
Pulmonary veins to atrial roof
disconnected from the atrioventricular junctions inferiorly, and

tilted superiorly. The pectinate muscles encircle the entirety of the
common atrioventricular junction, indicating the presence of
isomeric right atrial appendages. The common junction is guarded
by a valve with separate right and left atrioventricular valvar
orices.
Sup.
Right
Left
Inf.
Fig. 10.19 In this heart from a patient with isomeric right atrial
appendages, the pulmonary veins drain in anatomically anomalous
fashion to the atrial roof, even though returning directly to the
heart. Note that the common atrioventricular valve is connected
exclusively to a dominant left ventricle, indicating the presence of a
double-inlet connection.
junction1113. Interruption of the inferior

caval vein, with return through the azygos
venous system, is a frequent
accompaniment. In the more severely
affected cases, there may be a common

atrioventricular valve14. Pulmonary
stenosis or atresia is not usually a feature,
nor is the presence of univentricular
atrioventricular connection, but there is

frequently aortic coarctation.
Whatever the types of isomerism, when
there is a common atrioventricular valve
375
Morphologically
left atrial
appendage
Sup.
Ant.
Post.
Inf.
Fig. 10.20 The right-sided atrium in this heart has a

morphologically left appendage.
Morphologically
left atrial
appendage
appendage
Sup.
Ant.
Post.
Inf.
Fig. 10.21 The image shows the opposite side of the heart
illustrated in Figure 10.20. The left-sided appendage is also of left
morphology.
and each atrium is connected to its own

ventricle, it is frequent to nd a left-hand
pattern of ventricular topology. In this
setting, the patient may well have been
diagnosed as having congenitally corrected

transposition. The presence of isomeric
appendages, however, makes it highly
likely that there will be a sling of ventricular
conduction tissue joining dual

atrioventricular nodes (Figure 10.22, righthand panel)11. This places the entire edge
of the ventricular septum at risk should
376
Left bundle
branch in
left-sided
ventricle
Anterior
connection

Sling
Posterior
connection
Posterior
connection
surgical correction be attempted. When

isomerism of the appendages is found with
biventricular atrioventricular connections
and a right-hand pattern of ventricular
topology, the atrioventricular conduction
axis should be expected in its usual
posterior position (Figure 10.22, left-hand
panel). This entire discussion emphasises
the signicance of full sequential segmental
analysis of any patient presented for cardiac
surgery.
References
1. Wilkinson JL, Acerete F. Terminological
pitfalls in congenital heart disease. Reappraisal
of some confusing terms, with an account of a
simplied system of basic nomenclature. Br
Heart J 1973; 35: 11661177.
2. Van Praagh R, Weinberg PM, Matsuoka R,
Van Praagh S. Malpositions of the heart.
Edited by Adams FH, Emmanouilides GC.
In: Moss Heart Disease in Infants, Children
and Adolescents. Baltimore, MD: Williams &
Wilkins, 1983; pp 422458.
3. Byron F. Ectopia cordis. Report of a case
with attempted operative correction.
J Thorac Surg 1948; 17: 717722.
4. Cantrell JR, Haller JA, Ravitch MM. A

syndrome of congenital defects involving
the abdominal wall, sternum, diaphragm,
pericardium and heart. Surg Gynecol Obstet
1958; 107: 602614.
5. Anderson RH, Smith A, Wilkinson JL.
Disharmony between atrioventricular
connections and segmental combinations
unusual variants of criss-cross hearts. J
Am Coll Cardiol 1987; 10: 12741277.
6. Symons JC, Shinebourne EA, Joseph MC,
et al. Criss-cross heart with congenitally
corrected transposition: report of a case
with d-transposed aorta and ventricular
preexcitation. Eur J Cardiol 1977; 5:
493505.
7. Seo J-W, Yoo S-J, Ho SY, Lee HJ,
Anderson RH. Further morphological
observations on hearts with twisted
atrioventricular connections (criss-cross
hearts). Cardiovasc Pathol 1992; 1:
211217.
8. Stanger P, Rudolph AM, Edwards JE.
Cardiac malpositions: an overview based on
study of sixty-ve necropsy specimens.
Circulation 1977; 56: 159172.
9. Uemura H, Ho SY, Devine WA, Kilpatrick
LL, Anderson RH. Atrial appendages and
venoatrial connections in hearts with
orientation, shows the disposition of the axis of

atrioventricular conduction tissue in hearts
with isomerism of the atrial appendages,
biventricular atrioventricular connections, and
a common atrioventricular valve. The
arrangement varies depending on whether
there is right-hand ventricular topology (lefthand panel), when there is only a posterior
connection of the ventricular conduction
tissues, or left-hand topology (right-hand
panel), when the ventricular conduction tissues
are connected by both posterior and anterior
atrioventricular nodes, producing a sling of
conduction tissue along the crest of the
muscular ventricular septum.
10.
11.
12.
13.
14.
patients with visceral heterotaxy. Ann

Thorac Surg 1995; 60: 561569.
Uemura H, Ho SY, Devine WA, Anderson
RH. Analysis of visceral heterotaxy
according to splenic status, appendage
morphology, or both. Am J Cardiol 1995;
76: 846849.
Smith A, Ho SY, Anderson RH, et al. The
diverse cardiac morphology seen in hearts
with reference to the disposition of the
specialized conduction system. Cardiol
Young 2006; 16: 437454.
Dickinson DF, Wilkinson JL, Anderson
KR, et al. The cardiac conduction system
in situs ambiguous. Circulation 1979; 59:
879885.
Ho SY, Seo J-W, Brown NA, et al.
Morphology of the sinus node in human
and mouse hearts with isomerism of the
atrial appendages. Br Heart J 1995; 74:
437442.
Uemura H, Anderson RH, Ho SY, et al.
Left ventricular structures in
atrioventricular septal defect associated
compared with similar features with usual
atrial arrangement. J Thorac Cardiovasc
Surg 1995; 110: 445452.
Index
Numbers in italics refer only to illustrations
A
Abbotts artery 337, 338
absent pulmonary valve syndrome 239
accessory pathways 11419, 114
left-sided 116, 117
paraseptal 11719, 118
right-sided 117, 118
aneurysms
membranous septum 274
patent arterial duct 350
ansa subclavia 12
anterior septum 119
anterolateral muscle bundle hypertrophy 21617
aorta
anatomy 457
arch 456, 48
development 3401
double 3413
interrupted 3001, 33740
right-sided 3434
ascending 45, 47
coarctation 211, 3001, 3347
intercostal arteries 467, 336
common arterial trunk 3089, 312
congenitally corrected transposition 2816, 286
coronary arteries, anomalous origins 916, 313,
3545
descending 467
double-outlet ventricle 2956, 297, 3001
hypoplasia 334
parallel arterial trunks 31319
pulmonary arteries arising from 3456, 347
spiral arrangement of arterial trunks 313, 314
mirror-image 315
tetralogy of Fallot 2312
transposition 2758
vascular rings 3405
see also common arterial trunk
aortic atresia
in congenitally corrected transposition 291
with mitral atresia 260, 2636
aortic root 16, 52, 59, 91, 159
aortic sinuses 78, 914, 124, 21112
in aortic stenosis 218
Leiden nomenclature 93
in transposition 278, 280
aortic stenosis
subvalvar 210, 21318
dynamic 218
xed 21417
supravalvar 218, 21921
valvar 21013
bicuspid 21012, 21314

tricuspid 213, 214
unicuspid 210, 212
aortic valve
anatomy 45, 46, 53, 5560, 7381, 210
aorticmitral brous curtain 45, 56, 58, 79
conduction axis and 79, 82, 85, 112, 218
in double-outlet ventricle 2956, 297
endocarditis 218
imperforate 145
insufciency 210, 218, 2212
leaets 45, 46, 735, 757, 789
left coronary 79, 80
non-adjacent 79
right coronary 79
overriding 145, 197, 198
prolapse 195, 197, 198, 218, 223
sinutubular junction 45, 47, 52, 75, 77
stenosis see aortic stenosis
ventriculoarterial junction 52, 54, 77, 148, 149
zones of apposition 55, 79, 211
see also aortic atresia
aortopulmonary collateral arteries 224, 2324
aortopulmonary window 3513
aortoventricular tunnels 35961
arrhythmias
postoperative 1246, 260, 270
ventricular tachycardia 123
see also supraventricular tachycardia
arterial duct 9
in aortic coarctation 334, 336
in common arterial trunk 311
embryology 341
pulmonary arteries arising from 232, 233, 234,
345, 347
surgical closure 34851
in tetralogy of Fallot 232, 233, 234
arterial ligament 49, 347
arterial switch procedure 278, 27982
arterial trunk(s) 145
common see common arterial trunk
parallel 31317
discordant atrioventricular connections
31719
relationship to arterial valves 148
solitary 145, 303, 304
spiral arrangement 313, 314
mirror-image arrangement 315
arterial valves 525, 738, 148, 210
attachments/support 5560
malformations 145, 21041
see also aortic valve; pulmonary valve
atrial appendages 1617, 129, 130, 369
isomerism 19, 12932, 133, 143, 36976

anomalous pulmonary venous connection
132, 330, 369
juxtaposition 17
left see left atrium, appendage
right see right atrium, appendage
situs inversus 129
atrial brillation 1223, 126
atrial utter 1201
atrial maze operations 1246
atrial morphology
left 345
in malformed hearts 12932
right 1934
atrial septal defects 15562
oval fossa 22, 156, 1578
sinus venosus 1569, 1602, 3323
unroong of the coronary sinus 15962, 1634,
334
atrial septum 225, 27, 151, 152
atrioHisian tracts 114
atriopulmonary connection 2502
atrioventricular bundle (bundle of His) 59, 112,
11419, 189
in congenitally corrected transposition 280,
2856
in double-inlet ventricle 252
valvar anatomy and 79, 85
in ventricular septal defects 18791
atrioventricular conduction axis 2531, 11213
in atrial appendage isomerism 1301, 3756
in atrioventricular septal defects 16770, 1812,
1845
2856
when closing a ventricular septal defect 2856
in double-inlet ventricle
left dominant 252
right dominant 254
septation procedure 2523
ventricular septal defect enlargement 2478
in double-outlet ventricle 291, 294
Fontan procedure and 121, 1245
in parallel arterial trunk 319
in tetralogy of Fallot 226, 2289
in transposition 270
tricuspid valve 85
atresia (Fontan procedure) 125, 25960, 261
straddling 200, 201
valvar relationships 68, 79, 845, 86, 218
ventricular pre-excitation 11319
in ventricular septal defects 18791
see also sinus node
378
Index
atrioventricular groove 27, 118, 230

atrioventricular junction morphology in congenital
malformations 1329
biventricular and mixed atrioventricular 135,
143
concordant 134, 143
discordant 134, 143
uniatrial biventricular 136, 138, 143, 254, 258
univentricular 1369, 145
valves 1403
ventricular morphology 1323, 134, 143
ventricular relationships 1435
see also double-inlet ventricle
atrioventricular nodal tachycardia 119
atrioventricular node 256, 112, 113, 118
aortic valve and 79, 82, 85
artery 28, 31, 70, 89, 105, 118
in atrioventricular septal defects 168, 169, 185
mitral valve and 68, 69, 85
in transposition repair 270
atrioventricular septal defects 1556, 16685, 209
bridging leaets
morphology 1706
relationship to septal structures 1728
complete 172
conduction pathways 16770, 1812, 1845
Gerbode defects 34, 1645, 1657
hypoplasia of the ventricular septum 17981
intermediate 172, 177
left ventricular dominance 1845
left ventricular outow tract is narrow 1823
mural leaets 171
partial 172, 178
right ventricular dominance 1834
terminology 1624
ventricular shunting only 1756, 17880
atrioventricular septum
membranous see membranous septum
muscular 27, 151, 153
atrioventricular valves 52, 534, 556, 616
common 141, 1706, 174
atrial appendage isomerism 3734
common arterial trunk 306
double-inlet ventricle 246, 255
double-outlet ventricle 301
in congenitally corrected transposition
28991
malformations 199208
morphology in malformed hearts 1403
see also mitral valve; tricuspid valve
azygous vein 11, 12
with inferior caval vein abnormalities 324
pulmonary venous drainage via 32830
B
Bachmanns bundle 22
basal cords 63, 65, 68, 74
bicuspid valve see mitral valve
BlandWhiteGarland syndrome 353, 3545
brachiocephalic artery 12, 46

mirror-imaged 3445
brachiocephalic vein 2, 5, 109
pulmonary venous drainage via 328, 329
bronchial arteries 46, 47, 48, 350
bronchial obstruction 348
bundle of His see atrioventricular bundle
bundles of Kent 115
C
Cantrells syndrome 3656
cardiac position/malposition see under heart
cardiac veins 1057, 161
great 86, 87, 106, 107
middle 106
small 106
see also coronary sinuses
carotid artery, left 46
Carpentiers classication of mural leaet scallops
66, 68
caval veins
inferior
abnormalities 322, 323, 324
atrial appendage isomerism 132, 374
oval fossa and 156, 159
scimitar syndrome 330
sinus venosus and 156, 160
persistent left superior 8, 17, 18, 3223, 325
unroong of the coronary sinus 15960,
1612, 164
superior
abnormalities 322
proximity of phrenic nerve 3, 7
sinus venosus and 1569, 161
in venous switch procedures 267
total cavopulmonary connection 24850, 252, 259
cavoatrial junction 159
central brous body 313, 45, 579
ventricular septal defects 186
circumex coronary artery 71, 96, 1012, 105
valves and 70, 889
coarctation of the aorta 211, 3001, 3347
commissural cords 63, 64
commissures 55, 63
common arterial trunk 145, 148, 30313
arterial anatomy 30713
denition 303, 304
interventricular communications 3067
surgical repair 3045
truncal valve 303, 307, 3089
conduction axis see atrioventricular conduction axis
congenital malformations
cataloguing 1489
morphological analysis 129
atrial arrangement 12932
atrioventricular connections 1329
cardiac position 148
valves 1403, 145, 148
ventricular topology 1323, 134, 143
ventriculoarterial junctions 1458
surface anatomy 16, 17, 18, 19

see also specic conditions
congenitally corrected transposition 144, 27991,
366
complicating lesions 28291
conoventricular perimembranous defects 191
coronary arteries 91105
anomalies
classication 353
epicardial course 946, 3556, 358
stulous communications 3579
intramural course 355, 356
origins 916, 21112, 313, 3535
in common arterial trunk 310, 314
in double-outlet ventricle 301
interventricular
anterior 88, 96, 1001, 103, 366
inferior 89, 978, 100, 101, 105
left 19, 878, 99101
anomalies 92, 94, 353, 3545, 357, 360
atresia 355, 357
circumex 70, 71, 889, 96, 1012, 105
common arterial trunk 313, 314
dominance 70, 101, 105
intermediate 100, 103
main 99
myocardial bridging 102, 106
right 19, 39, 979
anomalies 92, 93, 94, 95, 353, 357, 359
dominance 978, 101
in tetralogy of Fallot 231
valves and 72, 89
valves and 879
mitral 70, 71, 889
tricuspid 72, 75, 89, 97, 98, 100
coronary sinuses 1067, 322
atresia 325, 327
in atrioventricular septal defects 16870
dilation 325
pulmonary venous drainage via 330, 331, 334
unroong 15962, 1634, 334
valvar relationships 70, 867, 8788
coronary veins 1057, 161
see also coronary sinuses
Cox maze III procedure 126
criss-cross heart 145, 3669
cysterna chyli 107
D
d-transposition 263, 276
DamusKayeStansel procedure 247
dead-end tract 112
dextrocardia 364
see also right-sided heart
diverticulum of Kommerell 343, 344
double aortic arch 3413
double-inlet ventricle 136, 24554
conduction axis 2478, 2523, 254
left dominant 136, 245, 24753
Fontan procedure 247, 24852
Index
Holmes heart 248, 315, 316

right dominant 246, 254
solitary/indeterminate ventricle 136, 139, 140,
254, 256
double-outlet ventricle 145, 146, 147, 225, 261,
292303
denition 2924
discordant atrioventricular connections 291, 294
left 303
nature of the interventricular communication
2949
surgical repair 297, 3023
double switch procedure 291
ductus arteriosus see arterial duct
E
Ebsteins malformation 117, 2024, 262
in congenitally corrected transposition 289, 290
ectopia cordis 3656
Eisenmengers syndrome 323
endocarditis of the aortic valve 218
epicardium 1416
Eustachian ridge 23, 29
Eustachian valve 156, 260, 323
exteriorisation of the heart 3656
hemitruncus 345
hepatic veins 322
Horners syndrome 12
hypoplastic left heart syndrome 140, 260,
2636
I
indeterminate ventricle 140, 254, 256
inferior caval vein see caval veins, inferior
infundibular artery 93, 99
infundibulum 38, 39, 41, 83, 151
absent in ventricular septal defect 196, 197
atretic 2356, 237
in double-outlet ventricle 297, 2989, 303
stenosis 300
interatrial groove see Waterstons groove
intercostal spaces
fourth 3, 12
fth 6
sixth 3, 8
intercostal veins 6, 8, 910
interrupted aortic arch 3001, 33740
interventricular arteries
anterior 88, 96, 1001, 103, 366
inferior 89, 978, 100, 101, 105
F
Fallots tetralogy see tetralogy of Fallot
brous tissue
aortic valve 79, 81
central brous body 313, 45, 579, 186
brous trigones 33, 56, 57
Fontan procedure
combined mitral and aortic atresia 2601
conduction axis and 121, 1245
double-inlet ventricle
left dominant 247, 24852
right dominant 254
tricuspid atresia 125, 25760, 261
foramen ovale see oval fossa
G
gastric veins 331, 333
Gerbode defects 34, 1647
Glenn procedure 162, 250
H
heart
malposition 364
criss-cross 145, 3669
exteriorisation 3656
right-sided 3478, 364, 366
position 2, 148
surface anatomy 14, 1619
K
Kochs triangle 258, 2930, 72, 112, 119, 152
Konno procedure 80, 83
L
laryngeal nerve see recurrent laryngeal nerve
lateral thoracotomy
left 36
right 612
latissimus dorsi 3
left atrium 345
access 15, 19, 34
appendage 17, 34, 36, 129, 130, 131
isomerism 19, 12930, 131, 132, 133, 36974
vestibule 35, 37
left bundle branch 45, 85, 112, 115
left ventricle 435
double-outlet 303
incomplete 254, 255
in univentricular atrioventricular connections
1378, 145
ventriculoarterial junction 52, 54
left ventricular dominance 136, 1845, 24753
Fontan procedure 247, 24852
left ventricular outow tract (subaortic outow

tract) 45
obstruction/stenosis 80, 83, 210, 21318
in atrioventricular septal defects 1823
Leiden convention (aortic sinus nomenclature)
91, 93
levoatrial cardinal vein 323, 326
ligament of Marshall 49
linea alba 2
lung
agenesis of the right lung 3478
arterial supply in tetralogy of Fallot 224, 2324
lymphatic system 6, 10, 10710, 337, 350
M
macro-reentrant atrial tachycardia 125
Mahaim connections 114, 119
median sternotomy 23, 5, 16
mediastinum 2, 3, 3
membranous septum 324, 45, 79, 151
aneurysm 274
ventricular septal defect 186
mitral atresia 137, 141, 199, 254, 258, 2601, 323
mitral valve
anatomy 53, 6170, 177
clefts 206, 208
conduction axis and 68, 69, 85
coronary arteries and 70, 71, 889
coronary sinuses and 70, 867
dilation 201
brous support 45, 56, 58, 59, 61
imperforate 1401, 261, 266
leaets 44, 612, 63, 667, 66, 171, 173
aortic 56, 64, 65, 66
mural 62, 65, 667, 68
offset with respect to tricuspid valve 26, 30
overriding 1423
papillary muscles 44, 68, 69, 200
parachute deformity 2078, 209
prolapse 64, 66, 205, 2068
straddling 142, 200
tendinous cords 62, 634, 678
zone of apposition 56, 667
see also atrioventricular valves; mitral atresia
moderator band 41, 147, 148, 153, 155
muscle of Lancisi (medial papillary muscle) 40,
70, 73
Mustard procedure 266, 26970
N
Nikaidoh procedure 275
nodal arteries
atrioventricular 28, 31, 70, 89, 105, 118
sinus see sinus node, artery
nodule of Arantius 74
non-branching bundle 181, 252, 280, 2856
non-coronary sinus 78, 92
Norwood procedure 247, 260
379
380
Index
O
oblique ligament 16
oblique sinus 1516
omphalocele 3656
ostium primum defects 1556, 16685, 209
ostium secundum defects see oval fossa, defects
outow tract tachycardias 123
oval fossa 23, 2728, 151, 152
defects 156
absent 158
decient 22, 157
perforated 157
P
papillary muscles
mitral valve 44, 68, 69, 200
tricuspid valve
anterior 41, 231
medial 40, 70, 73
parachute deformity
mitral valve 2078, 209
tricuspid valve 208
parallel arterial trunks (with concordant
ventriculoarterial connections) 31317
and discordant atrioventricular connections
31719
patent arterial duct 9
aortic coarctation 334, 336
surgical closure 34851
tetralogy of Fallot 232, 233
pectinate muscles 19, 22
atrial arrangements 129, 131132
penetrating atrioventricular bundle see
atrioventricular bundle (bundle of His)
pericardial cavity 1416
oblique sinus 1516
transverse sinus 1415, 45, 59, 68, 99
pericardial fold 15
pericardium 1416
phrenic nerve 23, 4, 5
left 6, 8, 46
right 6, 7, 7
portal vein 331, 3323
primary atrial septum (septum
primum) 151
pulmonary arteries
aortic origin 3456, 347
left 49
arising from right 3467, 3489
in pulmonary atresia with intact ventricular
septum 238
in pulmonary valve insufciency 23941
right 12, 4950, 347
spiral arrangement of arterial trunks 313, 314
mirror-image 315
total cavopulmonary connection 24850, 252,
259
trunk 45, 479, 145

aortopulmonary window 3513
atriopulmonary connection 2502
coronary artery arising from 353, 3545
dilated, in pulmonary valve insufciency
23941
solitary 145, 303, 304
see also common arterial trunk
pulmonary atresia
in congenitally corrected transposition 291, 292
with intact ventricular septum 2358, 260, 261
in tetralogy of Fallot (with ventricular septal
defect) 224, 2324
pulmonary hypertension 346
pulmonary stenosis 2246
subvalvar 210, 273
supravalvar 2214
valvar 21921
pulmonary valve
anatomy 55, 57, 738, 814
dysplasia 218, 224
imperforate 145, 2367, 238
insufciency 23841
leaets 38, 39, 735, 823
left adjacent 82
non-adjacent 82, 83
right adjacent 82, 83
overriding 145
ventriculoarterial junction 52, 54, 148, 149
see also pulmonary atresia; pulmonary stenosis
pulmonary veins 7
anomalous connection 157, 163, 32334, 369,
374
atrial brillation and 122
pulmonary venous sinus 35
R
Rastelli classication 1712, 174
Type A 174
Type B 175
Type C 175
Rastelli procedure 275
recurrent laryngeal nerve
closure of patent arterial duct 350
left 46, 9, 46
right 11, 12
right atrium 1934
access 19
appendage 17, 19, 129, 130, 131
anomalous pulmonary venous connection
132, 330, 369
isomerism 129, 130, 132, 133, 143, 369,
3702
pulmonary venous drainage to 330, 331
septal surface 225, 27
venous sinus 19, 20, 26

vestibule 34
right bundle branch 85, 112, 182, 191
right ventricle 19, 3543
double-outlet see double-outlet ventricle
incomplete
double-inlet ventricle 247, 248, 248, 252
tricuspid atresia 257, 259
in univentricular atrioventricular connections
1378, 145
ventriculoarterial junction 52, 54
right ventricular dominance 1834, 254
right ventricular outow tract
obstruction/stenosis 210, 218
see also infundibulum
right-sided aortic arch 3434
right-sided heart 3478, 364, 366
Ross procedure 84
S
scimitar syndrome 330
secondary atrial septum (septum secundum) 151
secundum defects see oval fossa, defects
semilunar valves see aortic valve; pulmonary
valve
Senning procedure 266, 270
septal band see septomarginal trabeculations
septal perforating arteries 42, 100, 104, 105
septomarginal trabeculations (septal band) 401,
42, 1478, 153, 226
in tetralogy of Fallot 226, 231, 232
in ventricular septal defect 1945
septoparietal trabeculation 41, 42
hypertrophy in tetralogy of Fallot 225, 229,
2301
see also moderator band
serratus anterior 3
Shones syndrome 208
sinus node 19, 21
artery
course 19, 202, 236, 36, 99, 101,
1023
oval fossa and 156, 158
preservation 34, 159, 251, 25960,
2667
variants 21, 23, 102, 1034, 106, 269
horseshoe 20, 21
isomerism of the atrial appendages
1301, 369
sinus venosus defects 1589, 163
transposition 266
sinus venosus defects 1569, 1602, 3323
sinutubular junction 45, 47, 52, 75, 77
pulmonary stenosis 219, 221
supravalvar aortic stenosis 218, 21921
Index
situs inversus 129

situs solitus 129
solitary arterial trunk 145, 303, 304
solitary origin of the coronary arteries 946, 97,
313, 355, 356357
solitary ventricle 136, 139, 140, 254, 256
Sondergaards groove see Waterstons groove
spleen 129, 369
strut cords 63, 65, 68
subaortic outow tract see left ventricular outow
tract
subclavian arteries 6, 10, 11, 46
development 341
interrupted aortic arch 339, 340
isolated 345, 346
subclavian sympathetic loop (ansa subclavia) 12
subpulmonary obstruction/stenosis 210, 218
sudden cardiac death 94, 355, 356
sulcus terminalis (terminal groove) 19, 20
superior caval vein see caval veins, superior
superoinferior ventricles 3669
supraventricular crest 389, 40, 83, 152,
155, 226
supraventricular tachycardia
atrial brillation 1223, 126
atrial utter 1201
atrioventricular nodal tachycardia 119
postoperative 1246, 260, 270
WolffParkinsonWhite syndrome
11419
surgical approaches 19
left-sided thoracotomy 36
right-sided thoracotomy 612
swiss-cheese ventricular septal defect 193
T
tachycardia see supraventricular tachycardia;
ventricular tachycardia
TaussigBing malformation 200, 297, 299, 3001
tendon of Todaro 25, 32, 59, 63, 112, 113
terminal crest 19, 22
terminal groove 19, 20
aortic connection 2312
conduction axis 226, 2289
left superior caval vein 323
pulmonary atresia 224, 2324
subpulmonary obstruction 22731
ventricular septal defects 226, 227, 228, 230
Thebesian valve 1067, 108, 323
thoracic artery, internal 2, 3, 7
thoracic duct 6, 10, 10710, 337, 350
thoracotomy
left-sided 36
right-sided 612
thymus gland 2, 3, 4, 45
arterial supply 2
venous drainage 2, 5
thyroid artery, inferior 2
tissue tags
aortic stenosis 217
subpulmonary obstruction
288, 289
total cavopulmonary connection 24850, 252, 259
trabeculations
comparison of left and right ventricles 43,
132, 134
left ventricle 445
right ventricle 3743
see also moderator band; septomarginal
trabeculations; septoparietal trabeculation
tracheo-oesophageal compression 342
transposition 26178
aorta 2758
with complicating lesions 2705
coronary arteries and the arterial switch
procedure 278, 27982
similarities in parallel arterial trunk 318
terminology 2614, 276
venous switch procedures 26670
see also congenitally corrected transposition
transverse sinus 1415, 45, 59, 68, 99
triangle of Koch 258, 2930, 72, 112, 119, 152
tricuspid atresia 137, 141, 199, 203, 251,
25460
tricuspid valve
anatomy 34, 616, 702
conduction axis and 85
Fontan procedure 125, 25960, 261
straddling valve 200, 201
coronary arteries and 72, 75, 89, 97, 98, 100
dilation 201
dysplasia 205, 206
Ebsteins malformation 117, 2024, 262
289, 290
brous annulus rare/absent 59, 62, 72
imperforate 1401, 142, 260, 2612
leaets 367, 389, 612, 63, 701
anteriosuperior 70
inferior 71
septal 70, 73, 190
offset with respect to mitral valve 26, 30
papillary muscles
anterior 41, 231
medial 40, 70, 73
parachute deformity 208
straddling/overriding 1423, 189, 199, 200,
2013
tendinous cords 62, 634, 70, 71, 74
in ventricular septal defect 189, 190, 199
transposition 2701, 2723
zones of apposition 37
see also atrioventricular valves; tricuspid atresia
truncal valve 303, 307, 3089
truncus arteriosus see common arterial trunk
381
U
univentricular atrioventricular connections 1369,
145
unroong of the coronary sinus 15962, 1634, 334
V
vagus nerve 2, 4, 5, 46
closure of patent arterial duct 350
valvar annulus, in echocardiography 55,
60, 77
valve of Vieussens 107
vascular pedicle 47
vascular rings 3405
vascular sling 3467, 3489
venae cavae see caval veins
venous duct 331
venous sinus
pulmonary 35
systemic 19, 20, 26
venous switch procedures 26670
ventricle(s) 1719
margins 1819
morphological analysis 1323, 134, 143
univentricular atrioventricular connections
1369, 145
solitary 136, 139, 140, 254, 256
superoinferior 3669
see also left ventricle; right ventricle
ventricular pre-excitation 11319
ventricular septal defects 18699
categorisation 186, 188
conduction axis 18791
291
large defects not to be confused with 247, 254,
257
doubly committed and directly juxta-arterial
187, 1958, 300
congenitally corrected transposition
286, 288
transposition 273, 274, 277
in interrupted aortic arch 339, 340
muscular 186, 1915
transposition 270, 272, 272, 273
perimembranous 18691, 199
congenitally corrected transposition 287
and doubly committed 198
transposition 270, 271, 272
in tetralogy of Fallot 226, 227, 228, 230
tricuspid valve and
atresia 260
cleft 190
382
Index
ventricular septal defects (cont.)

overriding/straddling 189, 199, 2701,
2723
ventricular septal hypoplasia 17981
ventricular septum 1515
ventricular tachycardia 123
ventriculoarterial junctions 523, 77
common see common arterial trunk
connections 145
infundibulum 1458
valvar/truncal relationships 148, 149

valves 145
ventriculoinfundibular fold 83, 147,
152, 155
ventricular septal defect
1945
vestibule
of mitral orice 35, 37
of tricuspid orice 34
visceral heterotaxy 129, 369
W
Warden operation 159
Waterstons groove (interatrial groove) 19, 22, 26,
28, 34, 35, 251
WolffParkinsonWhite syndrome 11419
X
xiphoid process 2

Wilcox's Surgical Anatomy of The Heart 4th Ed (PDF) (Tahir99) VRG PDF

Uploaded by

Document Information

Original Description:

Original Title

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

Wilcox's Surgical Anatomy of The Heart 4th Ed (PDF) (Tahir99) VRG PDF

Uploaded by

Copyright:

Available Formats

Wilcoxs Surgical Anatomy

Wilcoxs Surgical Anatomy

Diane E. Spicer, BS, PA(ASCP)

Andrew C. Cook, BSc, PhD

University Printing House, Cambridge CB2 8BS, United Kingdom

Surgical approaches to the heart

Anatomy of the cardiac chambers

Surgical anatomy of the valves of the heart

Surgical anatomy of the coronary circulation

Surgical anatomy of the conduction system

Analytical description of congenitally malformed hearts

Lesions with normal segmental connections

Lesions in hearts with abnormal segmental connections

Abnormalities of the great vessels

Positional anomalies of the heart

The books and articles devoted to

feedback from those who had used the rst

Diane Spicer to our anatomical team. She

obtained using computed tomography and

Illustrations and Photography, University

Wilcoxs Surgical Anatomy of the Heart

When we describe the heart in this chapter,

white line, or linea alba, is incised between

infant (Figures 1.3, 1.4). It has two lateral

Long axis of body

Fig. 1.1 The computed tomogram, with the

cardiac cavities delimited subsequent to

Surgical approaches to the heart

Fig. 1.2 This view, taken at autopsy,

sternotomy, therefore, with the nerve not

pericardial cavity may also lead to phrenic

demonstrates the anatomical relationships of

requiring greater degrees of exposure, the

Wilcoxs Surgical Anatomy of the Heart

Fig. 1.3 This view, taken at autopsy,

Fig. 1.4 This view, taken in the operating

Superior caval vein

require elevation when the incision is

and descending thoracic aorta, and the

room through a median sternotomy in an

which passes around the inferior border of

Surgical approaches to the heart

Fig. 1.5 This operative view, again taken through a median

Left vagus & recurrent laryngeal nerve

Left internal thoracic artery

Right vagus & recurrent laryngeal nerve

Fig. 1.6 As shown in this cartoon of a median

Wilcoxs Surgical Anatomy of the Heart

Left pericardiophrenic vein

Left phrenic nerve

Fig. 1.7 This operative view, taken through a

Right phrenic nerve

Fig. 1.8 This operative view, taken through a

posterior wall of the aorta, running adjacent

the phrenic and vagus nerves (Figures 1.11,

the duct, which is usually posteriorly

Surgical approaches to the heart

Fig. 1.9 This operative view, taken through a

Right pulmonary veins

Fig. 1.10 This operative view, taken through

Wilcoxs Surgical Anatomy of the Heart

Left phrenic nerve