When and How to Enlarge the Small Aortic Root

Phan-Kiet Trana,b and Victor Tsanga

Successful enlargement of the small aortic root in children has remained a management
challenge, particularly in the neonates and small infants. Achieving this aim requires thorough
understanding of the anatomic features of the left ventricular outow tract, careful patient
selection, and skilful execution of complex surgery. This article reviews the anatomical
principles upon which the surgical techniques rely, the decision-making, the timing of surgery,
the surgical options, and the outcomes.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 19:55-58 C 2016 Elsevier Inc. All rights
reserved.

Introduction
Left ventricular outow tract (LVOT) obstruction in neo-
nates, infants, and small children is one of the remaining
challenges to which there is no good surgical solution.
Although the LVOT is a very short channel, the anatomy,
mechanical function, and performance of the aortic root are
highly complex with perfect integration. To restore the
functional integrity in the case of surgery on the hypoplastic
The morphology of the aortic root and complex
aortic annulus, often surrounded by diseased LVOT, robust
topography of adjacent structures including, the
pre-operative work-up would be required to select those sinotubular junction, the sinus of Valsalva, the
suitable for timely aortic root/LVOT interventions. It is well tricuspid and mitral valves, the coronary arteries
accepted that the use of valve (and annulus) conserving and the conduction system.
operations to allow the children to grow, instead of applying
the Ross / Konno operation in early infancy, can minimise Central Message
risk.13 However, the real question of when to enlarge a small Understanding the anatomy and physiology of
aortic root, which is how small is too small, must be the aortic root is the key to successful
addressed. Its untimely delay can risk the development of enlargement.
circulatory failure and excessive ventricular hypertrophy.
It comes as no surprise that neonates/small infants with
congenital aortic stenosis with a hypoplastic aortic root and/or related technical modications including the concomitant
multiple levels of LVOT obstruction can carry many pitfalls, resection of endocardial broelastosis, which can benet these
such as a borderline left ventricle, the presence of endocardial children with a reconstructed, wide-open, and competent
broelastosis, smallish mitral valve, arch hypoplasia, and LVOT without growth limitation. This at least provides a
pulmonary hypertension. These are real challenges. On the robust physiological platform to address other important
other hand, the current root enlargement technique of choice is haemodynamic lesions as part of a staged management of
the Ross-Konno procedure, apart from some institutionally complex LVOT syndrome.

a
Department of Paediatric Cardiac Surgery, Great Ormond Street Hospital for
Children NHS Foundation Trust, London, UK.
b
Pediatric Heart Centre, Skane University Hospital, Lund, Sweden.
Dr Phan-Kiet Tran was supported by the Swedish Heart-Lung Foundation.
Address correspondence to Victor T. Tsang, MS, MSc, FRCS, Professor of
Cardiac Surgery UCL, Consultant Cardiothoracic Surgeon, Great Ormond
Street Hospital for Children NHS Trust, Great Ormond Street, WC1N 3JH
London, UK. E-mail: Victor.Tsang@gosh.nhs.uk
The Aortic Root is a Highly
Sophisticated Structure
The two main functions of the aortic root are: 1) to act as an
anchor for the synchronised movement of the inlet mitral valve
and outlet semilunar valve; and 2) to support the intricate
movements of the aortic valve cusps. The fact that our best

http://dx.doi.org/10.1053/j.pcsu.2015.11.007 PEDIATRIC CARDIAC SURGERY ANNUAL  2016 55

1092-9126/& 2016 Elsevier Inc. All rights reserved.
56
Figure 1 Illustration of the complex anatomy of the aortic root.
(Reprinted with permission from Anderson RH. The surgical anatomy
of the aortic root. Multimed Man Cardiothorac Surg 2007;2007
(102).10)
cardiac morphologists are still not in agreement on how to
dene the existence of the aortic annulus suggests that this is a
structure of exquisite design. The aortic annulus is best
described as crown shaped, and it is to this shape and structure
that the aortic valve is attached and supported in the root.4,5
For every heartbeat, studies applying cinematography and
contrast injections have visualized aortic root motion wherein
the root is displaced downward during systole and returns to
its previous position in diastole.6 Magnetic resonance imaging
(MRI) studies have elegantly revealed an axial downward
motion and a clockwise twist during systole.79 It has been
suggested that this motion retracts the aortic root into the
LVOT and presumably protects the root itself from unneces-
sary dilatation during systole.
The challenge for the surgeon is to understand this exquisite
morphology and function of the aortic root and then try to
surgically reproduce as many aspects of it as possible. In this
endeavour, the surgeon will also need to have a precise
understanding of the topography of adjacent structures,
including the sinotubular junction, the sinus of Valsalva, the
tricuspid and mitral valves, the coronary arteries, and the
conduction system (Fig. 1).10
Essential Pre-Operative Work-Up
Echocardiography
The cardiac ultrasound examination is the fundament of all
pre-operative work-ups. In this specic context, it can
determine the degree and impact of obstructions at multiple
levels along the entire LVOT. The aortic valve morphology,
size, and function can be assessed. A cardiac ultrasound can
also provide important assessments of the mitral valve size and
function, left ventricular volume, and the possible presence of
endocardial broelastosis. Associated malformations, includ-
ing arch hypoplasia or interruption and coarctation, and
P.-K. Tran and V. Tsang
pulmonary vascular issues such as pulmonary hypertension
can be identied. The management of neonates/small infants
with a borderline left ventricle can be very challenging in the
assessment of its adequacy to support a systemic circulation.11
MRI
In our experience, the main limitation of MRI is its availability
of expert acquisition of data and its interpretation, and then
there is the need of sedation or general anaesthesia in young
patients for quality imaging. MRI can otherwise provide
valuable ow and volume data to provide additional informa-
tion on the size of the left ventricle and its hemodynamic
performance. These data are particularly important in cases of
borderline left ventricle and presence of intra and extra cardiac
shunts, where the assessment of left ventricular systolic and
importantly diastolic function is difcult. In the absence of a
reliable non-invasive test, MRI combined with ultrasound may
increase the sensitivity to the diagnosis of endocardial broe-
lastosis. MRI can also provide additional information on
quality and size of the pulmonary valve.
Computer Tomography
Modern computed tomography (CT) examination is very fast
and normally does not require any sedation or general
anaesthesia. Its popularity is further enhanced with anatomical
and 3D reconstruction in complex associated malformations,
including total anomalous venous return, interrupted aortic
arch, and coarctation.
Cardiac Catheterization
In the presence of MRI and CT, conventional catheterization is
of limited value from a diagnostic point of view. However,
many newborn and younger children will have undergone a
conventional catheterization in a prior attempt to dilate the
critical aortic stenosis. This has become the main cause of aortic
regurgitation.
Decision-Making and Timing
In children with signicant aortic valve/annular hypoplasia, the
most important issue is to achieve effective delay of the
inevitable valve replacement without undue compromise of
the myocardial function. When the aortic root structures
display small dimensions with Z-score o -2.5 to -3.0,12 a
root enlargement procedure would be considered. The
Z- score would only be a guide and its measurement and
translation from real dimension can be skewed in very small
children. Obviously, the smaller the dimension the stronger is
the indication for root enlargement procedure. However, a too
small aortic root may, from a surgical technical point of view,
be non-permissive of safe coronary transfer and therefore
outweigh the advantages of the pulmonary autograft implan-
tation procedure. There may be some discrepancy between the
smallish aortic valve annulus and the larger pulmonary valve.
The latter is usable as long as the valve function does not
When and How to Enlarge the Small Aortic Root
display more than mild regurgitation in the presence of
elevated pulmonary arterial pressure.
Current literature provides conicting data on the timing of
surgery. The controversy lies in the risk of neo-aortic root/
autograft dilatation over time. Some data suggest that early
pulmonary autograft implantation into the systemic circulation
is associated with reduced dilatation over time. This is
speculated to be an effect of increased ability of the less
differentiated pulmonary valve and root to adapt and become
more aortic in nature.13 Other studies would point to an
indolent constant dilatation of the neo-aortic root regardless of
age at implantation.1416 These ndings at least suggest that the
length of time of pulmonary autograft exposure to high
systemic pressure is an important risk factor. Therefore,
Ross-Konno should be, within reason, deferred for as long as
possible. Of note, various wrapping techniques to prevent
dilatation would be counterproductive in growing children.
In reality, the decision on timing may not be that difcult,
because the patients most often belong to two separate entities.
We have on one hand the neonates and infants who will need
subacute root enlargements because of imminent circulatory
failure because of critical aortic stenosis and complex LVOT
obstruction, and on the other hand an elective operation for
toddlers and children with slow disease progression in terms of
limited physical performance and left ventricular hypertrophy.
In this latter group of patients, with a lower risk of planned
surgery, the timing may be determined by the second principle
defer to limit the time of exposure of pulmonary autograft to
high systemic pressure and reduce risk of excessive autograft
dilatation with time.
Surgical Options
Hypoplasia of the aortic valve annulus, diffuse obstructive of
the LVOT, and the lack of growth after prosthetic valve
insertion have led to the design of a number of procedures
aiming to enlarge the aortic root.17 The goals of any procedure
that aims to restore adequate function of a pathologically small
aortic root must include the relief of outow obstruction and
the restoration of valvar function. The latter requirement sets
the limit of what can be done because there are no good aortic
valve substitutes with a reasonable functional life span for small
children. In the aortic position, bio-prosthesis and aortic
homograft have proven to be short-lived. Mechanical valves
do not come in sizes smaller than 15 mm and they require
anticoagulation.
To date, the best option available for the growing infant and
child is the living pulmonary autograft combined with root
enlargement if required (Ross-Konno). The operation offers the
possibility of at least a medium life-time durability of the
autograft and is particularly useful in patients with a small
aortic root or multilevel LVOT obstruction.18,19
For older children and adolescents where larger aortic valve
prosthesis are within feasible range, root enlargement techni-
ques such as Nicks, Nunez or Manougian may be used.20 Most
congenital heart surgeons are well acquainted with the
technical aspects of these procedures.17
57
How to Complete the Ross-Konno
The Konno operation (aortoventriculoplasty) was developed in
the late 1970s to treat tunnel type of subaortic stenosis and
hypoplasia of the aortic valve annulus.2 The position of the
right coronary artery is accurately noted, and a longitudinal
aortotomy incision is made down to the aortic annulus well to
the left of the right coronary artery. A transverse incision is
made in the outow tract of the right ventricle, proximal to the
pulmonary annulus. An incision is made between the right
ventricle and the aorta, opening the interventricular septum.
This opening is extended far enough below the tunnel stenosis
if present. The incision stays above the medial papillary muscle
of the tricuspid valve to avoid the conduction system.
The aortic valve is completely removed and a valve sizer is
then laid in the area of the divided annulus to estimate the
amount of prosthetic patch required to enlarge the aortic
orice and to accommodate a predetermined valve size. An
oblong-shaped bovine pericardial or Gore-Tex patch is
trimmed and sutured to the ventricular septum by running
suture reinforced by horizontal mattress sutures on the right
ventricular side of the septum. The suturing of the patch is
interrupted just beyond the aortic annulus, and the appropri-
ately sized aortic valve prosthesis is then sutured in place in the
standard manner, with simple interrupted sutures or mattress
sutures through the aortic annulus and the newly created
valve ring.
There are a number of institutionally related technical
variations of the procedure. The main differences reside in
the choice of material to enlarge the interventricular septum
and the suturing technique.
The options for the patch include:
1. Gore-tex
2. Bovine pericardium
3. Extended harvest of the muscular RVOT together with
the pulmonary autograft.
Implantation of the pulmonary autograft can be accom-
plished by either interrupted (our preference) or a running
suture. At our institution, the suturing of the pulmonary
autograft onto the newly enlarged aortic annulus including the
septal patch is preferentially done with interrupted sutures into
the subannular position.17 The coronaries must be reim-
planted precisely and care should be taken to avoid injury to
the conduction system.
Results
Ross-Konno Procedure
A recent report from the Italian Ross Registry of data between
1990 and 2012 consisted of 305 children from 11 centres with
mid-term mean follow-up of 8.7 years; 73 of these had
undergone the Ross-Konno procedure. Early mortality was
27.6% for infants versus 0.2% for older children; the reason
was largely because of the very high requirement of Ross-
58
Konno in the infants group (two out of three if early surgery is
needed).3
Maeda and colleagues21 at Stanford reported excellent mid-
term outcome on the largest cohort of Ross-Konno. Between
1994 and 2010, a total of 24 patients o 1 year of age
underwent the modied Ross-Konno procedure. The LVOT
was enlarged with a right ventricular infundibular free wall
muscular extension harvested with the autograft. There was
one early death and no late mortality. Overall the 1-, 2-, and
5-year survival rate was 95% 4.5%. Freedom from aortic
stenosis was 94.7% 5.1% at 1, 2, and 5 years.21 Of note,
three deaths were not included in the report because of
borderline left ventricle. These deaths emphasize the impor-
tance of careful patient selection in the presence of suboptimal
substrates.
At our institution, a total of 87 Ross/Ross-Konno were
performed from 2004 to 2012, whereof nine were Ross-
Konno. There were two early and one late mortality, all had
undergone Ross-Konno. All four neonates (the oldest being 32
days) in the entire cohort required a Ross-Konno procedure
(unpublished data). One of the early mortality came from this
group of neonates because of persistent pulmonary hyper-
tension and our modest experience with borderline left heart
was recently reported.11
There are only limited long-term data for the Ross-Konno
procedure. Nelson and colleagues22 recently published results
of 240 children (mean follow-up, 10.7 years) that have
undergone the Ross procedure, whereof only three have had
Ross-Konno. Specic outcome of these three patients were not
reported in details.
Summary
If the Z-score of aortic annulus falls below -2.5 to -3.0, the need
for surgical intervention increases. The procedure of choice for
neonates/infants with critical aortic stenosis and multiple level
of LVOT obstruction is the Ross-Konno procedure. The patient
will benet greatly by meticulous preoperative work-up, a clear
understanding of the aortic root morphology, and the perfect
execution of surgery. Despite the creation of a patent and
competent LVOT, the outcome can still be skewed because of
borderline left ventricular volume and function, endocardial
broelastosis, and pulmonary hypertension.
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