AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

The term double outlet right ventricle (DORV) refers to a heterogeneous series of associated cardiac anomalies that are typically characterized by a partial ventriculoarterial connection in which both of the great arteries are associated with the right ventricle, including an aortic override of 50% or more. The anatomic dysmorphology of DORV can vary from that of tetralogy of Fallot (TOF) on one end of the spectrum to complete transposition of the great arteries (TGA), or transposition of the great vessels (TGV), on the other end. 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.

History of the Procedure

The first successful biventricular repair for this entity was reported by Sakakibara et al in 1967.

Problem

The definition of a DORV has been a point of controversy among professionals in the field of congenital heart surgery. The aorta and main pulmonary artery are typically dextrorotated from their usual positions and, as such, they are side by side (or nearly so). With rotation of the great vessels, discontinuity (typically aortomitral discontinuity) can be observed between the atrioventricular valve and the semilunar valve. All exceptions are related to the associated TGA.

In general, from a surgical perspective, defining the lesion as DORV 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. Subaortic and subpulmonary infundibula, as opposed to a subaortic conus, are often present in the setting of TGA. From an embryologic view, derangement of abnormal development of the bulboventricular loop occurs; therefore, derangement of conal rotation and conal absorption is present.

Essential features that differentiate TOF, DORV, and TGA include conal rotation, conal absorption, and development of the bulboatrioventricular ridge.

Frequency

In the United States, the incidence of DORV is 0.09 cases per 1000 live births.

Etiology

No specific causal agent or predictive event has been identified.

Clinical

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, such as rales, rhonchi, and wheezing, as well as 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

Section 3 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

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 DORV 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.

Regardless of the type of DORV present, most authors prefer to perform repair during infancy.

RELEVANT ANATOMY

Section 4 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Before 1972, 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, and he proposed that aortomitral fibrous discontinuity was required.¹ In addition, Lev et al began to classify the group of anomalies in DORV by the location of the ventriculoseptal defect (VSD) (ie, the great vessel to which the VSD was anatomically adjacent). DORV is almost always associated with a VSD. Lev et al noted 4 possibilities of commitment of the VSD 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 DORV and on surgical considerations.

The relative anatomic anomalies identified in the spectrum of DORV determine the clinical presentation and the surgical approach required for repair. DORV can be described in terms of the relative position of the great arteries and the relative position of the VSD.

TGA variations include the following:

• Side by side (most common type)
• Dextromalposition
• Levomalposition
• Normal (rare type)

VSD variations include the following:

• Subaortic (most common)
• Subpulmonic
• Doubly committed (uncommon)
• Uncommitted (uncommon)

VSDs are typically large and nonrestrictive.

Several associated cardiac anomalies are associated with DORV. 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%

CONTRAINDICATIONS

Section 5 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Absolute contraindications of double outlet right ventricle (DORV) repair include clinically significant left ventricular hypoplasia, an overriding atrioventricular valve, or Rastelli type C straddling of the atrioventricular valve. In those patients who are unsuitable for biventricular repair, single ventricle palliation would be indicated.

WORKUP

Section 6 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Lab Studies

• Specific laboratory testing during an evaluation of double outlet right ventricle (DORV) is not indicated.
• However, during preoperative evaluation, routine laboratory tests are appropriate, including the following:
• • CBC count
  • Electrolyte levels: In the setting of congestive heart failure and its management, monitoring and correction of variances are appropriate.
  • Coagulation panel: Assessment of bleeding time is not indicated.
  • Baseline nutrition laboratory values (eg, prealbumin, transferrin): In a patient with failure to thrive, optimal caloric intake is often absent. In the patient with a suboptimal nutritional status, these laboratory results can prompt enteral or supplemental feeding to improve the patient's clinical status and lower the risk of perioperative morbidity.

Imaging Studies

• Echocardiography of the heart and great vessels
• • 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.
  • Echocardiography can be used to successfully diagnose 95% of cases of DORV and define the 16 categories of relevant anatomic variations.
  • Sanders et al (1982) reported that standard transthoracic echocardiography was used to diagnose conotruncal malformation in 109 of 113 infants.² Of the 12 infants in whom DORV was diagnosed and confirmed with angiography, 11 previously received a diagnosis based on subxiphoid 2-dimensional 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 DORV but is not yet a routine or well-established diagnostic modality for DORV. MRI is useful to obtain additional anatomical information such as relationship of both ventricles.
• Chest radiography
• • Regardless of the end of the clinical spectrum (TOF or TGA) at which DORV 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 Taussig-Bing 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 DORV.
• Common findings in a child with DORV 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.

TREATMENT

Section 7 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Medical Therapy

Medical management in the treatment of double outlet right ventricle (DORV) is based on the underlying physiology. A spectrum of presentations is possible with DORV, ranging from inadequate pulmonary blood flow with associated hypoxia and cyanosis to torrential pulmonary blood flow with congestive heart failure. However, DORV 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

As stated above, DORV is a disorder that cannot spontaneously resolve, and the diagnosis alone is a sufficient indication for surgery. When DORV repair is planned, several anatomic and physiologic factors are reviewed. The location of the VSD and its size are critical to the repair.

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 DORV 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, pericardium, polytetrafluoroethylene [PTFE], Dacron; DuPont, Wilmington, DE) 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. This enlargement can be accomplished through the tricuspid valve or right ventricle with a transverse or longitudinal right ventriculotomy. 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. Placing the first suture through the base of the tricuspid valve leaflet (ie, anteroseptal commissure) and then through the mid portion of the tube graft is helpful. Approximately one third of the VSD sutures are placed along the posterior and inferior rim of the defect through the tricuspid valve; care is taken to avoid the conduction tissue, with several of these pledgetted sutures on the atrial side of the septal leaflet of the tricuspid valve. The VSD sutures are passed through the patch, which is seated down, and the sutures are tied. The remainder of the circumference of the VSD is closed through the right ventriculotomy; proper orientation of the patch is always maintained.

Some advantage may be gained by anchoring a portion of the pledgetted sutures through the anterior wall of the right ventricle above the aortic valve, similar to how this is done in a Rastelli procedure. 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 of autologous pericardium to prevent right ventricular outflow tract obstruction.

Repair of double outlet right ventricle - Subaortic ventriculoseptal defect and pulmonary stenosis

Establishing an adequate right ventricular outflow tract without a valved conduit is sometimes possible, as is done with repair of TOF. In this case, after the obstructing right ventricular muscle bundles are divided, a transannular outflow tract patch is created by using autologous pericardium. If the pulmonary valve annulus is of adequate size, an infundibular patch is adequate after pulmonary valvotomy is performed. Otherwise, a transannular patch is necessary.

In patients who have DORV with subaortic VSD and pulmonary stenosis, identifying the coronary arteries and marking the planned right ventriculotomy incision with stay sutures before cardioplegic arrest is effected is important. Intraventricular tunnel repair of the VSD is identical to that used for patients who have DORV and subaortic VSD without pulmonary stenosis. If a coronary artery crosses the right ventricular outflow tract or if pulmonary vascular resistance is elevated, a valved conduit should be used to create continuity of the right ventricle and pulmonary artery.

After the pulmonary trunk is divided, the main pulmonary artery is oversewn. The proximal portion of a valved homograft is sewn to the superior aspect of the right ventriculotomy. The homograft is trimmed to the proper length, and an end-to-end anastomosis is created between the distal homograft and the distal main pulmonary artery. If the main pulmonary artery is small, the conduit can be anastomosed to the pulmonary bifurcation. The gap between the proximal right ventriculotomy and the proximal homograft is bridged with leftover homograft material or autologous pericardium. In actual practice, just the anterior surface is patched because the use of patch extensions (ie, tube grafts) is a risk factor for stenosis.

Anatomic repair of double outlet right ventricle with subpulmonary ventriculoseptal defect

The preferred surgical repair of DORV 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 DORV can be accomplished. Not all subpulmonic VSDs are Taussig-Bing anomalies. The VSD must be subpulmonic, and the great vessels must be in a side-by-side arrangement. If the orientation is anteroposterior, the criteria are not met.

The VSD is closed through a right atriotomy or through a right ventriculotomy channeling left ventricular blood into the pulmonary artery. The aorta is transected slightly higher than the pulmonary trunk.

Particularly with side-by-side great arterial relationships, the circumflex coronary artery often arises with the right coronary artery from the right posterior-facing sinus, whereas the anterior descending coronary artery originates from the left anterior-facing sinus. In this situation, the anterior descending coronary artery is excised as a button and is transferred to a defect created in the left posterior-facing sinus of the proximal pulmonary trunk (ie, neoaorta). The right and circumflex coronary ostium is excised as a large button. It is transferred above the right anterior-facing sinus of the proximal pulmonary trunk (ie, neoaorta) and is incorporated into the suture line between the proximal pulmonary trunk (ie, neoaorta) and the distal ascending aorta. Positioning this coronary button more superiorly as it is transferred to the left reduces potential kinking of the circumflex coronary artery.

When the great vessels are side by side, reconstruction of the right ventricular outflow tract can lead to compression of one of the coronary arteries or predispose the right ventricular outflow tract to external obstruction (if the repair is performed exactly as is typical for TGA). This can be caused by overrotation or discrepancy between the original proximal ascending aorta (ie, neopulmonary artery) and the distal pulmonary trunk.

The surgeon can address this issue by opening the right lateral aspect of the distal pulmonary trunk onto the undersurface of the right pulmonary artery after performing the Lecompte maneuver, bringing the pulmonary bifurcation anterior to the distal ascending aorta. The U-shaped defect in the neopulmonary valve (ie, site of coronary ostial buttons) is repaired with a patch of treated autologous pericardium. The resulting increase in the circumference of the proximal neopulmonary artery accommodates the typical size discrepancy between the aorta (smaller) and the pulmonary trunk (larger). This patch, incorporated into the incision on the undersurface of the right pulmonary artery, results in a tension-free pulmonary arterial anastomosis.

As an alternative, with side-by-side great arteries, the pulmonary artery can be reconstructed by using the same techniques outlined above without incorporating the Lecompte maneuver. To do this, transection of the aorta higher than the pulmonary trunk at the beginning of the repair is important.

Intraventricular repair of double outlet right ventricle with subpulmonary ventriculoseptal defect

In patients with DORV and subpulmonary VSD with side-by-side great arteries, intraventricular tunnel repair (ie, Kawashima procedure) is feasible.

The subpulmonary VSD is exposed through a transverse right ventriculotomy. The VSD must often be enlarged anteriorly and superiorly in relationship to the conduction system. The infundibular septum between the aorta and pulmonary artery is usually prominent and can cause subaortic obstruction before surgery. This septum is excised to provide an unimpeded channel from the left ventricle through the VSD to the aorta. The interventricular tunnel then is created by using an opened PTFE tube graft, as is done with a DORV and subaortic VSD.

If the relationship of the great arteries is more anteroposterior than side by side, the distance between the tricuspid valve and the pulmonary valve is inadequate. The intraventricular tunnel obstructs the subpulmonary region, and a switch with VSD-to–pulmonary artery repair or a Rastelli-type reconstruction of the right ventricular outflow tract is required. In this situation, an arterial switch–based procedure is the preferred option.

Damus-Kaye-Stansel repair of double outlet right ventricle with subpulmonary ventriculoseptal defect and subaortic stenosis

Some patients who have DORV and subpulmonary VSD also have clinically significant subaortic stenosis that may not be amenable for successful resection. Thus, this condition precludes an arterial switch operation because postoperative obstruction of the right ventricular outflow tract results. In this clinical situation, the Damus-Kaye-Stansel procedure is an acceptable surgical option.

With the Damus-Kaye-Stansel procedure, the subpulmonary VSD is closed to direct left ventricular blood through the pulmonary valve. This can be approached through the right atrium or through a right ventriculotomy incision.

Transect the pulmonary trunk just proximal to the pulmonary bifurcation. Distortion of the proximal pulmonary trunk or ascending aorta must be avoided so that semilunar valve insufficiency does not result. Placing a marking suture on the medial aspect of the proximal pulmonary trunk, the planned site of the transection, is useful. Along the left medial aspect of the ascending aorta, corresponding marking suture is placed to define the proximal extent of the aortic incision and to ensure proper orientation of the great arteries.

Open the aorta along the left medial aspect from the previously placed marking suture for a length that corresponds to the diameter of the pulmonary trunk. Carefully identify the coronary arteries and avoid any distortion. Perform an end-to-side anastomosis between the proximal pulmonary trunk and the left medial ascending aorta with a patch. Use a homograft valved conduit to create right ventricle–to–pulmonary artery continuity.

In the Damus-Kaye-Stansel procedure, the aortic valve remains connected to the right ventricle. Because aortic pressure is always higher than right ventricular pressure, the aortic valve remains closed. Right ventricular output is directed through the valved conduit into the (low pressure) pulmonary artery.

Aortic valvular insufficiency is poorly tolerated. Regurgitant blood flow is directed into the right ventricle, creating a left-to-right shunt, and can be misdiagnosed as a residual VSD. In the setting of aortic insufficiency, the aortic valve can be oversewn.

Repair of double outlet right ventricle with doubly committed ventriculoseptal defect

Surgical correction of DORV with a doubly committed VSD (an uncommon variant of this disorder) is performed in a fashion similar to that described above for DORV 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–to–pulmonary artery conduit.

Repair of double outlet right ventricle with noncommitted ventriculoseptal defect

Of the types of DORV, 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 DORV 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 DORV is more of a malposition of the aorta than anything else, 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.

Absolute contraindications to performing a biventricular repair include the following:

• Significant left ventricular hypoplasia
• Overriding atrioventricular valve
• Rastelli type C straddling of the atrioventricular valve

However, the following are not contraindications to biventricular repair:

• Restrictive VSD
• Tricuspid conal chordae
• Reduced pulmonary-tricuspid distance
• Infundibular stenosis

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 infundibuli 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.^{3, 4}

When the VSD is distant, tunnel repair can be dangerous because it can cause a functionally important area of akinesis in the left ventricular outflow tract. As the length of the tunnel increases, the incidence of late and clinically significant subaortic stenosis increases.

When the VSD is situated in the inlet septum, Lacour-Gayet (2001) advocates using a tunnel to connect the VSD to the ostium infundibuli, followed by an arterial switch procedure.⁵ 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

Section 8 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

See Intraoperative Details for discussion.

OUTCOME AND PROGNOSIS

Section 9 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

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%.⁶ Brown et al reported that 15-year survival rates after repair for noncomplex DORV 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.

FUTURE AND CONTROVERSIES

Section 10 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Consensus surgical therapeutic strategies are available for noncomplex tetralogy type double outlet right ventricle (DORV) and Taussig-Bing anomaly. Controversy remains as to whether or not the indication of biventricular repair should be extended to borderline anatomic subgroups, such as small left-sided structures (including mitral valve and left ventricle) or nonsubaortic VSD. The Subgroups who have these risk factors have suboptimal early and long-term outcomes. Furthermore, significant improvement of survival and quality of life in single ventricle palliation and subsequent Fontan completion makes this issue debatable.

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

1. Lev M, Bharati S, Meng CC, et al. A concept of double-outlet right ventricle. J Thorac Cardiovasc Surg. Aug 1972;64(2):271-81. [Medline].
2. Sanders SP, Bierman FZ, Williams RG. Conotruncal malformations: diagnosis in infancy using subxiphoid 2- dimensional echocardiography. Am J Cardiol. Dec 1982;50(6):1361-7. [Medline].
3. Barbero-Marcial M, Tanamati C, Atik E, Ebaid M. Intraventricular repair of double-outlet right ventricle with noncommitted ventricular septal defect: advantages of multiple patches. J Thorac Cardiovasc Surg. Dec 1999;118(6):1056-67. [Medline].
4. Belli E, Serraf A, Lacour-Gayet F, et al. Double-outlet right ventricle with non-committed ventricular septal defect. Eur J Cardiothorac Surg. Jun 1999;15(6):747-52. [Medline].
5. Lacour-Gayet F. Double outlet right ventricle with uncommitted VSD. Presented at: 81st Annual Meeting of the American Association for Thoracic Surgery; May 6, 2001; San Diego, CA.
6. Brown JW, Ruzmetov M, Okada Y, Vijay P, Turrentine MW. Surgical results in patients with double outlet right ventricle: a 20-year experience. Ann Thorac Surg. Nov 2001;72(5):1630-5. [Medline].
7. Anderson RH, McCarthy K, Cook AC. Continuing medical education. Double outlet right ventricle. Cardiol Young. May 2001;11(3):329-44. [Medline].
8. Bradley TJ, Karamlou T, Kulik A, Mitrovic B, Vigneswaran T, Jaffer S. Determinants of repair type, reintervention, and mortality in 393 children with double-outlet right ventricle. J Thorac Cardiovasc Surg. Oct 2007;134(4):967-973.e6. [Medline].
9. Chin AJ, Yeager SB, Sanders SP, et al. Accuracy of prospective two-dimensional echocardiographic evaluation of left ventricular outflow tract in complete transposition of the great arteries. Am J Cardiol. Mar 1 1985;55(6):759-64. [Medline].
10. Delius RE, Rademecker MA, de Leval MR, et al. Is a high-risk biventricular repair always preferable to conversion to a single ventricle repair?. J Thorac Cardiovasc Surg. Dec 1996;112(6):1561-8; discussion 1568-9. [Medline].
11. Judson JP, Danielson GK, Puga FJ, et al. Double-outlet right ventricle. Surgical results, 1970-1980. J Thorac Cardiovasc Surg. Jan 1983;85(1):32-40. [Medline].
12. Kanter KR. Double outlet ventricles. In: Kaiser LR, Kron IL, Spray TL, eds. Mastery of Cardiothoracic Surgery. Philadelphia, PA: Lippincott-Raven; 1998.
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Article Last Updated: Jun 4, 2008