Author

Abdullah A Alghamdi, MD, FRCSC Fellow, Division of Pediatric Cardiac Surgery, University
of Toronto, Hospital for Sick Children
http://emedicine.medscape.com/article/904397-treatment#showall
The term double outlet right ventricle (DORV) refers to a heterogeneous series of associated
cardiac anomalies that involve the right ventricular outflow tract in which both of the great
arteries arise entirely or predominantly from the right ventricle. The anatomic dysmorphology of
double outlet right ventricle can vary from that of tetralogy of Fallot (TOF) on one end of the
spectrum to complete transposition of the great arteries (TGA) on the other end (see the image
below).

Double outlet right ventricle (DORV) with transposition of the great

arteries accounts for 26% of cases of DORV. The aorta (AO) is anterior and to the right of the
pulmonary artery (PA), and both arteries arise from the right ventricle (RV). The only outflow
from the left ventricle (LV) is a ventricular septal defect (VSD), which diverts blood toward the
RV. Pulmonary veins drain into the left atrium (LA) after blood has been oxygenated in the lungs
(L). Systemic venous return is to the right atrium (RA).
The clinical presentation can vary from one of profound cyanosis to that of fulminant congestive
heart failure. As a result, management and surgical repair of the defect are based on correcting
the specific combination of anatomic defects with their radically different pathophysiologies.
Although hearts with atrioventricular discordance (ie, congenitally corrected TGA) or
univentricular atrioventricular connections (ie, double inlet left ventricle) can be correctly
grouped in this spectrum of anomalies, this article focuses on only those hearts with
atrioventricular concordance and 2 functional ventricles

Problem
The definition of a double outlet right ventricle has been a point of controversy among
professionals in the field of congenital heart surgery. However, the Congenital Heart Surgery
Nomenclature and Database Project defines double outlet right ventricle as a type of
ventriculoarterial connection in which both great vessels arise either entirely or predominantly
from the right ventricle.[2, 3]

In general, from a surgical perspective, defining the lesion as double outlet right ventricle is
reasonable when more than 50% of both of the great arteries arise from the right ventricle. All of
one vessel and most of the remaining vessel typically arise from the right ventricle. From the
morphological standpoint, some suggest that the absence of the fibrous continuity between the
arterial and atrioventricular valves is a feature of double outlet right ventricle.

Epidemiology
Frequency

In the United States, the incidence of double outlet right ventricle is 0.09 cases per 1000 live
births. Double outlet right ventricle comprises about 1-1.5% of all congenital heart disease.[4]

Etiology
No specific causal agent or predictive event has been identified.

Pathophysiology
Based on the location of ventriculoseptal defect (VSD) in relation to great arteries, double outlet
right ventricle can be classified into 4 main categories: double outlet right ventricle with
subaortic VSD, double outlet right ventricle with subpulmonary VSD, double outlet right
ventricle with doubly committed VSD, and double outlet right ventricle with noncommitted
VSD.
Subaortic ventriculoseptal defect

This variant is most common. The pathophysiology depends on the degree pulmonary stenosis.
With pulmonary stenosis, the pulmonary blood flow is decreased with variable degrees of
cyanosis (TOF type). In the absence of pulmonary stenosis, the pulmonary blood flow is
increased, resulting in heart failure (VSD type).
Subpulmonary ventriculoseptal defect

In this variant of double outlet right ventricle, the pulmonary artery preferentially receives left
ventricular oxygenated blood, and the desaturated blood from the right ventricle streams to the
aorta (TGA type). The Taussig-Bing anomaly is a typical example of double outlet right ventricle
with subpulmonary VSD. Aortic arch hypoplasia is a common association.
Doubly committed ventriculoseptal defect

The infundibular septum is absent leaving both aortic and pulmonary valves related to the VSD.
Clinical features depend on the presence or absence of pulmonary stenosis.
Noncommitted ventriculoseptal defect

The noncommitted VSD is remote from the aortic and pulmonary valves. Most patients with
noncommitted VSD undergo single ventricular palliative strategies.

Presentation

Clinically significant cardiac anomalies might first be based on a complete history of the patient's
condition and its progression from the parents. Feeding tolerance, weight gain, breathing
problems, and a general failure to thrive should be elucidated.
Complete physical examination should include an evaluation of the cardiac valvular sounds, any
murmurs and thrills, the point of maximal impact, and heaving of the chest wall. In addition,
abnormal pulmonary signs (eg, rales, rhonchi, wheezing) and peripheral signs of cyanosis and
capillary refill should be sought.
The severity or lack of pulmonary stenosis largely determines the spectrum of symptoms and the
patient's age at the time of clinical presentation. In general, most patients present during the
neonatal period. Patients with severe pulmonary stenosis have cyanosis, and those with
uncontrolled pulmonary blood flow present with congestive heart failure.

Indications
Because double outlet right ventricle (DORV) is a disorder that cannot spontaneously resolve,
the diagnosis alone is a sufficient indication for surgery. In general, palliative operations are
performed only in patients who require short-term treatment, whereas noncardiac disease is
managed (eg, sepsis) when anatomic features do not allow for definitive correction.
In the ideal case, repair of double outlet right ventricle is a corrective operation that leads to
biventricular repair; thus, the left ventricle is connected to the aorta, and the right ventricle is
connected to the main pulmonary artery. Palliative operations differ on the basis of the
physiology of the subtype. In the case of excessive pulmonary blood flow, banding of the
pulmonary artery can be used to palliate excessive pulmonary flow and protect the pulmonary
vascular bed until definitive management can be undertaken. In the case of inadequate
pulmonary blood flow, an aortopulmonary shunt, typically a Blalock-Taussig shunt, can be used
to palliate inadequate pulmonary flow and promote growth for the pulmonary vascular bed and
acceptable oxygenation until definitive management can be undertaken.

Relevant Anatomy
Before 1972, double outlet right ventricle (DORV) was defined as complete emergence of both
great arteries from the right ventricle and no fibrous valvular continuity. The evaluation of this
entity by Lev et al (1972) altered how this was classified; the group proposed that aortomitral
fibrous discontinuity was required.[5] In addition, Lev et al began to classify the group of
anomalies in double outlet right ventricle by the ventriculoseptal defect (VSD) location (ie, the
great vessel to which the VSD was anatomically adjacent).
Double outlet right ventricle is almost always associated with a VSD. Lev et al noted 4
possibilities of commitment of the double outlet right ventricle to the great arteries and termed
them subaortic, subpulmonic, doubly committed, and noncommitted (or remote). The location of
the VSD has important implications on the physiologic manifestations of double outlet right
ventricle and on surgical considerations.

The relative anatomic anomalies identified in the spectrum of double outlet right ventricle
determine the clinical presentation and the surgical approach required for repair. Double outlet
right ventricle can be described in terms of the relative position of the great arteries and the
relative position of the VSD. In double outlet right ventricle, the associated VSD is typically
large and nonrestrictive.
Several associated cardiac anomalies are associated with double outlet right ventricle. Many of
these affect the clinical presentation and the limits of the repair. Occurrence rates of associated
cardiovascular anomalies are as follows:

Pulmonary stenosis - 21-47% (most commonly observed with subaortic type VSD)
Atrial septal defect - 21-26%

Patent ductus arteriosus - 16%

Atrioventricular canal - 8%

Subaortic stenosis - 3-30%

Coarctation, hypoplastic arch, and interrupted aortic arch - 2-45%

Mitral valve anomalies - 30%

Laboratory Studies
Routine laboratory testing in patients with double outlet right ventricle (DORV) includes CBC
count, electrolyte levels, renal profile, hepatic function, coagulation profile, and assessment of
nutritional status if indicated.

Imaging Studies

Echocardiography of the heart and great vessels

o Echocardiography generally provides enough information for accurate and
adequate diagnosis and provides the needed information to plan the surgical
approach in neonates and young infants.
o

Sanders et al reported that standard transthoracic echocardiography was used to

diagnose conotruncal malformation in 109 of 113 infants.[6] Of the 12 infants in
whom double outlet right ventricle was diagnosed and confirmed with
angiography, 11 previously received a diagnosis based on subxiphoid 2dimensional echocardiography.

Echocardiography can be used to correctly identify the relative position of the

great arteries, the degree of subsemilunar narrowing, the position of the VSD, and
the status of the mitral valve and left ventricle.

Cardiac angiography

Cardiac catheterization, once the criterion standard for confirming DORV, is now
rarely required in the evaluation or preoperative planning of this cardiac disorder.

Angiography has several advantages, when indicated.

In the older child with long-standing disease, hemodynamic parameters

can be directly measured.

In the setting of possible aberrant coronary anatomy (3-5% of cases), the

actual anatomic variation can be defined. This knowledge can affect
surgical planning when Rastelli-type repair is contemplated.

In the setting of pulmonary vascular anomalies, angiography can help in

defining the main pulmonary branches, the pulmonary vascular tree, and
the collateral vessels to the lungs.

MRI: This has been used in the diagnosis of double outlet right ventricle but is not yet a
routine or well-established diagnostic modality for double outlet right ventricle. MRI is
useful to obtain additional anatomical information such as relationship of both ventricles.

Chest radiography
o

Regardless of the end of the clinical spectrum (tetralogy of Fallot [TOF] or

transposition of the great arteries [TGA]) at which double outlet right ventricle
occurs, findings on anteroposterior and lateral chest radiography depend on the
degree of pulmonary (or subpulmonary) stenosis.

In the setting of severe stenosis, the pulmonary parenchyma is relatively oligemic,

whereas in the setting of minimal pulmonary stenosis (especially with a TaussigBing heart), findings are likely to be consistent with congestive heart failure.
Either way, the chest image shows cardiomegaly.

Other Tests

ECG findings are rarely diagnostic for double outlet right ventricle.
Common findings in a child with double outlet right ventricle include right ventricular
hypertrophy, right axis deviation, and, occasionally, evidence of left ventricular
hypertrophy.

Diagnostic Procedures

No additional procedures are indicated.

Histologic Findings

No particular histologic findings have been reported.

Staging

No staging has been applied for this anomaly.

Medical Therapy
Medical management in the treatment of double outlet right ventricle (DORV) is based on the
combination of anatomical lesions and the underlying physiology. A spectrum of presentations is
possible with double outlet right ventricle, ranging from inadequate pulmonary blood flow with
associated cyanosis to excessive pulmonary blood flow with congestive heart failure. However,
double outlet right ventricle is a disorder that cannot spontaneously resolve, and the diagnosis
alone is a sufficient indication for surgery.
In the setting of inadequate blood pulmonary blood flow, preserving ductal (ie, patent ductus
arteriosus) blood flow is vital. An infusion of prostaglandin E (ie, alprostadil) is the standard of
care until repair can take place. When the contrary clinical picture of congestive heart failure is
present, careful dieresis, digoxin, inotropic support, and control of pulmonary blood flow by
means of intubation and manipulation of blood gases may be indicated.

Surgical Therapy
When double outlet right ventricle repair is planned, several anatomic and physiologic factors are
reviewed. The location of the ventriculoseptal defect (VSD) and its size are critical to the repair.[7,
8, 9]

Preoperative Details
Preoperative studies should be used to accurately determine the surgically relevant features,
including the following:

Separation of the pulmonary valve from the tricuspid valve relative to the
diameter of the aortic valve annulus
Location of the VSD, including degree of involvement of the conal septum

Chordal attachments to the conal septum, VSD ridge, and presence of

straddling chordae

Degree of subpulmonary stenosis and whether it is fixed or dynamic

Degree of pulmonary valvar stenosis

Coronary anatomy

Relative size of the great vessels and their relationship

The aortic arch and the presence of coarctation

Intraoperative Details
Repair of double outlet right ventricle with subaortic ventriculoseptal
defect

Repair of double outlet right ventricle with a subaortic VSD is accomplished by creating an
intraventricular tunnel that channels left ventricular blood through the VSD to the aorta. This is
facilitated by the use of a patch (eg, polytetrafluoroethylene [PTFE]) that corresponds to the
circumference of the aorta.
After cardiopulmonary bypass with bicaval cannulation and cardioplegic arrest is established by
routine means, the intracardiac anatomy is carefully inspected through a right atriotomy. The
VSD is visualized through the tricuspid valve, and its relationship to the aorta is confirmed. If the
VSD is suspected to be smaller than the aorta before or during surgery, the VSD is enlarged. The
VSD is enlarged superiorly and anteriorly; thus, some of the infundibular septum is resected. The
conduction tissue runs inferiorly and is avoided.
The patch is oriented along its longitudinal axis corresponding to an imaginary line from the
anterior most portion of the aorta to the anterior-inferior limit of the VSD.
The VSD can be closed by using interrupted pledgetted sutures or a continuous-suture technique.
If the intraventricular tunnel appears to be bulging into the right ventricular outflow tract, the
right ventriculotomy is closed with a patch (eg, autologous pericardium) to prevent right
ventricular outflow tract obstruction.
In patients who have double outlet right ventricle with subaortic VSD and pulmonary stenosis,
the general approach is similar to those with tetralogy of Fallot (TOF). Identifying the coronary
arteries and marking the planned right ventriculotomy incision (if needed) before cardioplegic
arrest is effected is important. The obstructive right ventricular muscle bundles are divided. The
VSD is enlarged if restrictive.
Intraventricular tunnel repair of the VSD is similar to that used for patients who have double
outlet right ventricle and subaortic VSD without pulmonary stenosis. The pulmonary valve, main
pulmonary artery, and branch pulmonary artery are inspected and are patched as needed to
eliminate obstruction. If an important coronary artery crosses the right ventricular outflow tract,
a conduit may be added as an additional outflow path from the right ventricle. In patients with
crossing coronary arteries, palliative surgery may result in 2 right ventricular outflow tracts:
through the native pulmonary valve, and, if necessary, through the right ventricle to pulmonary
artery conduit.
Anatomic repair of double outlet right ventricle with subpulmonary
ventriculoseptal defect

The preferred surgical repair of double outlet right ventricle with subpulmonary VSD (ie, the
Taussig-Bing heart) is anatomic repair (ie, the arterial switch operation). Because coarctation of
the aorta is commonly observed in this situation, patients may have undergone coarctation repair
with a pulmonary artery band, although single-stage repair of the coarctation and double outlet
right ventricle can be accomplished. Atrial septectomy is performed if the atrial septum is
restrictive. The subsequent procedure is a single stage complete repair with VSD enlargement if

restrictive, repair of the VSD to direct the left ventricular blood to the pulmonary artery, followed
by an arterial switch procedure. When an aortic arch obstruction is also associated, it is repaired
at the same time under hypothermic circulatory arrest.
Repair of double outlet right ventricle with doubly committed
ventriculoseptal defect

Surgical correction of double outlet right ventricle with a doubly committed VSD (an uncommon
variant of this disorder) is performed in a fashion similar to that described above for double
outlet right ventricle with subaortic VSD. The VSD, which is typically large, usually does not
create difficulty in channeling left ventricular blood to the aorta with an intraventricular tunnel.
Concurrent pulmonary stenosis or obstruction of the right ventricular outflow tract due to the
tunnel may necessitate the creation of a right ventricle outflow patch or even a right ventricle
topulmonary artery conduit.
Repair of double outlet right ventricle with noncommitted ventriculoseptal
defect

Of the types of double outlet right ventricle, the defect that requires repair of the noncommitted
VSD is the most difficult to correct. Its correction is a high-risk procedure that often involves
univentricular repair. However, biventricular repair of double outlet right ventricle with
noncommitted VSD, based on specific anatomic features, is a challenging but achievable
outcome.
The major feature of this anomaly is a persistent subaortic conus and a double infundibulum. The
subaortic conus is in excess to essentially normal right ventricular structures. Therefore, this
variation of double outlet right ventricle represents malposition of the aorta, with a normally
positioned pulmonary artery and with the great vessels usually side by side. The VSD, usually
perimembranous, often has inlet and/or trabecular extension and can be restrictive. Crucial to
biventricular repair is the distance between the tricuspid and mitral annuli because the aortic
tunnel is constructed in this area.
Variations without pulmonary stenosis first require palliation with pulmonary artery banding.
Severe subaortic obstruction, restrictive VSD, or aortic arch obstruction requires palliation with
the pulmonary artery banding. Variations with stenosis may be physiologically palliated, or a
systemic-to-pulmonary shunt, such as a modified Blalock-Taussig shunt, may be required.
Contraindications to performing a biventricular repair include significant left ventricular
hypoplasia, major overriding, or straddling of the atrioventricular valve
With the use of combined atrial and ventricular approaches, an intraventricular tunnel that
connects the VSD to the aorta is the operation of choice. A right vertical infundibulotomy is
performed through the subaortic infundibulum, and the abnormal subaortic band between the
subaortic conus and conal septum is resected. The diameter of the VSD is measured through the
tricuspid valve and compared with that of the aorta, and the distance between the tricuspid
annulus and the ostium infundibulum is measured. This last measurement should allow for a
patch or tunnel that is at least the diameter of the aorta. Tricuspid chordal attachments blocking
the channel are detached and reimplanted on the patch, and the VSD is enlarged anteriorly by
avoiding the conduction tissue.

Two groups of investigators reported a mortality rate of approximately 10%, but the incidence of
late subaortic stenosis is reasonably high.[10, 11]
When the VSD is distant, tunnel repair is associated with clinically significant subaortic stenosis
in the early postoperative phase due to geometric errors in the construction of the tunnel and in
the late postoperative phase due to fibrous obstruction.
When the VSD is situated in the inlet septum, Lacour-Gayet advocates using a tunnel to connect
the VSD to the ostium infundibuli, followed by an arterial switch procedure.[12] He notes that the
perimembranous VSD is close and requires a smaller tunnel. Also, the creation of a tunnel to the
pulmonary artery does not depend on the pulmonary-tricuspid distance and is usually not
affected by the presence of conal tricuspid chordae located above the tunnel. The subaortic band
is resected, an infundibulotomy is created on the subpulmonary infundibulum, and the VSD is
expanded. Then, an arterial switch procedure is performed as described above. He reported
excellent early results, with resolution of patients' New York Heart Association (NYHA) status.

Postoperative Details
See Intraoperative Details for discussion.

Follow-up
See Intraoperative Details for discussion.

Complications
See Intraoperative Details for discussion.

Outcome and Prognosis

In a large series analyzing outcomes in patients with double outlet right ventricle (DORV) from
1980-2000, Brown et al reported that the 15-year overall survival rate (including the patients
who underwent no surgical intervention) is 56%.[13] Brown et al reported that 15-year survival
rates after repair for noncomplex double outlet right ventricle and for Taussig-Bing anomaly
were 95% and 89%, respectively.
Small left-sided structure, including left ventricle and mitral valve, structural abnormality of the
mitral valve, and aortic arch obstruction, has been identified as a risk factor for death after repair.