Ann Thorac Surg 2010;89:537-543. doi:10.1016/j.athoracsur.2009.10.049
© 2010 The Society of Thoracic Surgeons
Original Articles: Pediatric Cardiac
Biventricular Repair of Atrioventricular Septal Defect With Common Atrioventricular Valve and Double-Outlet Right Ventricle
Eric J. Devaney, MDa,*,
Timothy Lee, BAa,
Sarah Gelehrter, MDb,
Jennifer C. Hirsch, MDa,
Richard G. Ohye, MDa,
Robert H. Anderson, MD, FRCPathc,
Edward L. Bove, MDa
a Department of Surgery, Division of Cardiac Surgery, University of Michigan School of Medicine, Ann Arbor, Michigan
b Department of Pediatrics, Division of Pediatric Cardiology, University of Michigan School of Medicine, Ann Arbor, Michigan
c Cardiac Unit, Institute of Child Health, University College, London, United Kingdom
Accepted for publication October 21, 2009.
* Address correspondence to Dr Devaney, Department of Surgery, University of Michigan, 5143 Cardiovascular Center, 1500 E Medical Center Dr, Ann Arbor, MI 48109 (Email: edevaney{at}umich.edu).
Presented at the Fifty-fifth Annual Meeting of the Southern Thoracic Surgical Association, Austin, TX, Nov 5–8, 2008.
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Abstract
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Background: The combination of an atrioventricular septal defect with a common atrioventricular junction guarded by a common valve, and double-outlet right ventricle, is a rare lesion that presents a challenge for surgical repair. This report describes our surgical approach and results in 16 patients undergoing biventricular repair for such a combination of lesions.
Methods: A retrospective analysis was performed for all patients undergoing biventricular repair of atrioventricular septal defect with common atrioventricular valve and double-outlet right ventricle between 1991 and 2008. Patients with tetralogy of Fallot and common atrioventricular valve were excluded from analysis. Early and actuarial outcomes were evaluated using the 
2 test for categorical variables and Wilcoxon rank sum for ordinal variables.
Results: The median age at operation was 16 months. Heterotaxy syndrome was present in 12 of the 16 patients (9 right isomerism and 3 left isomerism), and 6 had concurrent totally anomalous pulmonary venous connections. Primary repair was achieved in 6 patients, and 10 underwent one or more prior operations (most frequently a shunt, banding of the pulmonary trunk, or repair of the anomalous pulmonary venous connections). Enlargement of the ventricular septal defect by resection of the muscular outlet septum was required in 11 patients, in whom the ventricular septal defect emptied entirely or primarily to the inlet of the right ventricle. A conduit was placed from the right ventricle to the pulmonary arteries in 13. There was 1 death before discharge from hospital, 1 late death, and 2 episodes of heart block. Among survivors, follow-up was complete with a median follow-up of 66 months. No patient had late obstruction of the left ventricular outflow tract. The presence of heterotaxy with totally anomalous pulmonary venous connections was associated with combined mortality and significant morbidity (p = 0.008).
Conclusions: Although technically challenging, the surgical repair can be accomplished with acceptable early results. Heterotaxy syndrome, with concurrent anomalous connections of the pulmonary veins, represented the strongest identified risk factor for death or significant complication.
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Introduction
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The combination of an atrioventricular septal defect with a common atrioventricular junction guarded by a common valve with double-outlet right ventricle (Fig 1) is a rare cardiac malformation for which surgical repair is particularly challenging due to the complex anatomy, including the remote nature of the interventricular communication, and the frequent associated defects including pulmonary stenosis, and anomalous pulmonary and systemic venous connections as seen with heterotaxy syndromes. Because of the high risk associated with complete repair, functionally univentricular palliation has been recommended by some centers [1, 2]. We have adopted a strategy of staged or primary complete repair for this lesion. In this report, we describe our experience with 16 such patients undergoing biventricular repair from 1991 to 2008.
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Fig 1. Anatomic features of atrioventricular septal defect with common atrioventricular valve and double-outlet right ventricle.
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Patients and Methods
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Data Collection and Analysis
All patients undergoing biventricular repair of double-outlet right ventricle seen in combination with a common atrioventricular junction guarded by a common valve were identified through the University of Michigan Congenital Heart Surgery database. A retrospective analysis of patient, procedural, and hospital course data was performed through a review of paper and electronic medical records. Follow-up data were obtained from pediatric cardiology clinic notes, or from communication with the referring cardiologist. Institutional Review Board approval was obtained before the initiation of this study. Data were recorded on a Microsoft Excel spreadsheet (Microsoft, Redmond, WA), and analysis was performed using Statview version 5.0.1 (SAS Institute, Cary, NC). Multiple risk factors were assessed individually by univariate analysis (2 for categorical variables and Wilcoxon rank sum for ordinal variables), and a p value less than 0.05 was used to determine significance.
Operative Procedures
All patients undergoing biventricular repair are placed on cardiopulmonary bypass with direct cannulation of the caval veins and moderate hypothermia. Deep hypothermia with low-flow cardiopulmonary bypass or brief circulatory arrest is utilized during the repair of totally anomalous pulmonary venous connection, when present, to improve exposure. After arrest of the heart, exposure of the common atrioventricular valve is achieved through either a right- or left-sided atriotomy, depending on anatomy. Repair of the defect is initially analogous to a two-patch repair of atrioventricular septal defect. A crescent shaped patch of polytetrafluoroethylene is used to close the inlet component of the interventricular communication, starting at the midpoint of the defect, and carrying the suture line inferiorly and posteriorly to the junction of the muscular ventricular septum with the annulus of the common atrioventricular valve (Fig 2). The anterior and superior portion of the patch is left unsutured. Marking stitches are placed in the patch superiorly where it would join the atrioventricular valvar annulus, and at the midpoint of the inlet component of the ventricular defect. Next, the corresponding portions of the superior and inferior bridging leaflets are secured to the crest of the patch, and the zone of apposition between the left ventricular components of the bridging leaflets is closed with fine interrupted sutures.
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Fig 2. Transatrial repair is performed initially, leaving the superior portion of the ventricular septal defect patch unclosed. Marking sutures are placed at the superior edge of the patch at the atrioventricular valve annulus and at the midportion of the inlet defect.
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The atrial septum is reconstructed using an autologous pericardial patch, which is tailored to accommodate the systemic and pulmonary venous connections. In the presence of heterotaxy syndrome, the frequent association of bilateral superior caval veins, separate hepatic venous and inferior caval venous entry into the heart, and anomalies of pulmonary venous connection, can make this patch complex. In these situations, separate atrial patches may be used to avoid twisting or obstruction when one or more of the venous orifices is located on the opposite side of the appropriate atrioventricular valve. A right ventriculotomy is made, and the previously placed marking sutures are transposed from the atrium through the ventriculotomy (Fig 3). Typically, enlargement of the interventricular communication is necessary because the defects open primarily or entirely to the inlet of the right ventricle, making them remote from the aortic valve. This is accomplished by resection of the muscular outlet septum, or infundibular septum, along with the subaortic infundibulum if present, beginning at the unsutured edge of the prosthetic patch, and extending it to the attachments of the leaflets of the aortic valve (Fig 4). A second patch of polytetrafluoroethylene is then trimmed (Fig 4), and sutured to the free edge of the original patch. An unobstructed intraventricular baffle is thereby constructed to connect the morphologically left ventricle to the aortic valve (Fig 5). In patients with pulmonary stenosis or atresia, division of septoparietal trabeculations in the right ventricular outflow tract is performed, or else a conduit is placed from the right ventricle to the pulmonary arteries.
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Fig 3. Exposure of the partially closed ventricular septal defect by a cut-away view through the right ventricle. Note that the previously placed marking sutures have been transposed though the ventriculotomy.
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Fig 4. Resection of the outlet muscular septum creates a wide communication with the left ventricle. A polytetrafluoroethylene patch is trimmed for placement of an intraventricular baffle.
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Fig 5. The intraventricular baffle is sewn in place to connect the ventricular septal defect to the aortic valve.
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Results
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Since March of 1991, 16 patients, 6 male and 10 female, have undergone biventricular repair of the combined lesions at C. S. Mott Children's Hospital at the University of Michigan. The profiles of the patients before the operative procedure are shown in Table 1. The median age at the time of operation was 16 months (range, 6 to 63). The common atrioventricular valve was deemed to be balanced in 13 patients, and mildly or moderately unbalanced to the right or left in the other 3. In all instances, the superior bridging leaflet was free floating, and extended significantly into the right ventricle, producing the Rastelli type C arrangement, with the exception of one, which was indeterminate. Preoperatively, moderate-to-severe atrioventricular valvar insufficiency was present in 2 patients, 3 had moderate insufficiency, and the remainder had mild or less.
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Table 1 Patient Profiles for 16 Patients Undergoing Biventricular Repair of Atrioventricular Septal Defect With Common Atrioventricular Valve and Double-Outlet Right Ventricle
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Double-outlet right ventricle was defined by the origin of both great arteries from the morphological right ventricle, and by the presence of a muscular subaortic infundibulum. This definition excluded patients with anatomy typical for tetralogy of Fallot. The interventricular communication opened mostly to the inlet of the right ventricle, albeit with some outlet extension in 9 patients. In all 9, however, a variable amount of subaortic infundibular muscle was present separating the leaflets of the aortic valve from the defect itself. In the remaining 7 patients, the interventricular communication opened exclusively to the inlet of the right ventricle. Thus, when seen in the operating room, all patients were deemed to possess a remote and noncommitted interventricular communication. The arterial trunks were arranged in side-by-side fashion in 4 (with the aorta to the right), positioned with the aorta anteriorly and to the right in 6, and with the aorta anteriorly and to the left in 6. Significant pulmonary stenosis was present in 13 patients, albeit iatrogenic in 3, after banding of the pulmonary trunk in 1, and division of the pulmonary trunk in 2 patients with systemic-to-pulmonary shunts. Heterotaxy syndrome was a common finding, seen in 12 patients, with right isomerism diagnosed in 9 cases and left isomerism in 3. Mirror-imaged atrial arrangement, with mirror-imagery of the remaining bodily organs, was observed in 1 patient, and mirror-imaged arrangement of the venous returns in another. Other associated defects occurred in all but 2 patients, and are listed in Table 2. Extracardiac totally anomalous pulmonary venous connection occurred in 6 patients with right isomerism, with 5 patients having supracardiac connections, and 1 having an infracardiac connection. In the remaining 3 patients with isomeric right atrial appendages, the pulmonary veins drained directly to the heart, albeit in anatomically anomalous fashion. Right-sided position of the heart was a complicating factor in 6 cases. Left-hand topology (L-looping) was present in 7 patients.
A complete primary repair was accomplished in 6 patients, and the remainder underwent one or more palliative procedures. In 7 patients, a systemic-to-pulmonary shunt was created, 3 in conjunction with repair of totally anomalous pulmonary venous connections, with 1 additionally requiring patch augmentation of the pulmonary arteries. Isolated repair of totally anomalous pulmonary venous connection was performed in 2 patients. The patient with infracardiac pulmonary venous connection subsequently had localized pulmonary venous stenosis, and this was addressed at the time of biventricular repair. One patient underwent banding of the pulmonary trunk. In 1 patient, a Fontan circulation had been constructed by means of a total cavopulmonary connection.
Biventricular repair was successfully accomplished in all 16 patients. The median cardiopulmonary bypass time was 242 minutes, with a median cross-clamp time of 158 minutes. Enlargement of the interventricular communication was required in 11 patients. A conduit was placed from the right ventricle to the pulmonary arteries in 13 patients, and 1 other patient with distortion of the pulmonary arteries subsequent to banding had the right ventricular outflow tract patched with a monocusp valve. The conduit was selected on the basis of size and availability, with bovine jugular venous xenografts implanted in 7 cases, pulmonary allografts in 4, an aortic allograft in 1, and a porcine heterograft in 1. Heart block developed postoperatively in 2 patients, who required implantation of a pacemaker. Tachyarrhythmias were common postoperatively, with 8 patients having transient junctional ectopic tachycardia. There has been 1 early death, which was secondary to pulmonary vascular obstructive disease, and 1 late death at 3 months due to complications related to renal failure. In addition, 2 surviving patients experienced significant early morbidity. One patient required extracorporeal membrane oxygenation postoperatively because of severe hypoxemia. A second patient had a prolonged stay in intensive care due to dependence on a ventilator and hydrocephalus. At the time of discharge, 5 patients had moderate atrioventricular valvar insufficiency, this being mild or trivial in the remainder. No patients had atrioventricular valvar stenosis, significant residual interventricular communications, or obstruction of the left ventricular outflow tract.
At a median follow-up of 66 months, with a range from 1 month to 17 years, all discharged patients were clinically well, in the first or second classes of the New York Heart Association functional class system. Reoperation has been necessary in 6 patients. The patient with the monocusp patch ultimately required insertion of a pulmonary valve 8 months after biventricular repair. This patient also underwent concomitant mitral and tricuspid valvoplasty. An additional patient has required replacement of the left atrioventricular valve, and implantation of a pacemaker 3 months after repair. Replacement of the conduit has been performed in 2 patients to date. Another patient had partial dehiscence of the patch closing the interatrial defect, which required reoperation. An unusual patient, who had previously undergone a modified Fontan procedure before biventricular repair, required reoperation for placement of an absorbable band on the left pulmonary artery owing to the presence of significant left-sided pulmonary arteriovenous malformations. A subsequent catheterization in this patient revealed resolution of the arteriovenous malformations and no pulmonary artery stenosis. At follow-up, 3 patients had mild-to-moderate left or right atrioventricular valvar insufficiency, or both, and the remainder had mild or less insufficiency. No patient had late obstruction of the left ventricular outflow tract.
A series of clinical characteristics were evaluated to determine risk factors for poor outcome. The only significant risk factor for mortality and significant morbidity was the presence of totally anomalous pulmonary venous connection (p = 0.008). Nonsignificant factors included age at operation, prior procedure, heterotaxy syndrome without anomalous pulmonary venous connections, form of isomerism, and presence of dextrocardia.
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Comment
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An atrioventricular septal defect with common atrioventricular junction guarded by a common valve and associated double-outlet right ventricle is an uncommon lesion. In one study of 507 pathological specimens having atrioventricular septal defect with common atrioventricular valve, the prevalence of double-outlet right ventricle was 6.7% [3]. Already in 1975, it had been suggested that the combination of lesions could be repaired surgically by enlarging the interventricular communication combining standard repairs for the other lesions [4]. Successful repair was first reported in 1980, but the mortality was high, especially in the presence of associated defects such as pulmonary stenosis or anomalous pulmonary venous connections [5]. Improving results have since been reported by several groups [6–10].
The repair differs from that for tetralogy of Fallot associated with common atrioventricular valve primarily because of the exclusively right ventricular origin of the aorta, with its valve supported by a muscular infundibulum. That creates the difficulty of redirecting the outflow from the left ventricle through the interventricular communication to the aortic valve. Even when the communication opens toward the outlet regions, it is frequently difficult to avoid obstruction of the newly created channel from the left ventricle without enlarging the interventricular communication. In these cases, some groups have recommended functionally univentricular palliation. Recently, the outcomes have been described for nearly 100 patients with complex double-outlet right ventricle, either associated with heterotaxy or a common atrioventricular valve [2]. In this series, only 8 patients underwent biventricular repair, whereas 88 were managed using the functionally univentricular strategy. The authors identified atrioventricular valvar regurgitation, pulmonary venous obstruction, and neonatal presentation as risk factors for mortality. The decision to proceed with univentricular palliation for atrioventricular septal defect with common atrioventricular valve and double-outlet right ventricle must be tempered by the understanding that many patients may be poor candidates for the Fontan procedure owing to pulmonary artery stenosis, pulmonary hypertension, pulmonary venous obstruction, atrioventricular valve insufficiency, or other factors. In fact, 1 patient in our series had previously undergone Fontan palliation but was referred for conversion to biventricular repair because of chronic hypoxemia, caused by unilateral pulmonary arteriovenous malformations that developed as a consequence of diversion of hepatic venous blood away from that lung by the existing cavopulmonary connection.
We identified heterotaxy in 12 of our patients. This diagnosis was generally made by the surgeon or cardiologist, using a number of criteria including splenic anatomy, bronchopulmonary anatomy, venoatrial connections, and morphology of the atrial appendages. The most accurate method of determining the isomerism, which is the best means of stratifying heterotaxy, is by analysis of the pectinate musculature of the atrial appendage, but this cannot generally be determined by echocardiography, and is not always assessed by the surgeon in the operating room [11–13]. Both forms of isomerism are associated with a spectrum of complex cardiac malformations, and the complete surgical repair or palliation of these defects is accompanied by an extremely high mortality rate [14, 15]. Additionally, right isomerism is always linked to anomalous pulmonary venous connection, even if the pulmonary veins return directly to the heart, as such connections cannot be anatomically normal. The repair is often complicated by the development of pulmonary venous stenosis, which carries a poor prognosis [16]. We also found that the anomalous pulmonary venous connections associated with the heterotaxy syndrome proved to be a risk factor for poor outcome.
In our series, early and late obstruction within the newly created subaortic outflow tract were avoided by aggressive enlargement of the interventricular communication, which was required in most cases. Intraoperative echocardiography and direct intraventricular pressure measurement were utilized to assess the repair and adequacy of the left ventricular outflow tract. Transection of the aorta to examine the left ventricular outflow tract was not used in this series but may be useful in selected difficult cases. As illustrated in Figure 4, resection of the muscular outlet septum and subaortic infundibular musculature creates a wide communication with the left ventricle. In those patients with left-hand topology, or L-looping, the pathway of the conduction system would be expected to lie in the septum that is being resected, resulting in unavoidable complete heart block, although this was seen in only 1 patient in this series. A ventriculotomy is generally required to provide adequate visualization for this maneuver and, owing to the frequent occurrence of pulmonary stenosis in these patients, the right ventriculotomy is also necessary for placement of a conduit. Others reporting biventricular repair for this combination have similarly emphasizes the need for enlargement of the interventricular communication into the muscular outlet septum [10, 17].
The technical complexity of complete repair in these patients is frequently further complicated by the need to place a complex patch to separate the systemic and pulmonary venous return. In the presence of heterotaxy syndrome, bilateral superior caval veins, separate drainage of the hepatic and inferior caval veins, and anomalous pulmonary venous connections all serve to make septation of the atrioventricular channels challenging. Use of autologous or allograft pericardium facilitates the placement of a Mustard-like patch with the atrium, and obstruction to venous return has been rare in our patients. In patients with low pulmonary blood flow, the atrial volume is frequently small, further compromising repair.
Although this series represents the largest reported group of patients undergoing biventricular repair of the combined lesions, the rarity of the lesion still results in a relatively small number of patients, and extensive statistical analysis is not possible. We submit, nonetheless, that our results support the approach of biventricular repair, even in the presence of complex venous connections and a remote interventricular communication. Although the rate of reoperation will be high, particularly for replacement of the conduit, it is hoped that this approach will offer long-term benefits when compared with functionally univentricular palliation. More extensive follow-up of this cohort of patients will be important to justify this strategy.
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Discussion
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DR JAMES A. QUINTESSENZA (St. Petersburg, FL): I would like to congratulate you, Dr Devaney, and colleagues from the University of Michigan on an excellent report of your institution's experience with 16 patients undergoing biventricular repair of atrioventricular septal defect/double-outlet right ventricle (AVSD/DORV), not to be confused with AVSD/tetralogy of Fallot, which are distinctly different. This is a relatively rare lesion, as you pointed out. It occurs in 6% of AVSD cases, and this subgroup presents with a high incidence of heterotaxy and totally anomalous pulmonary venous connection (TAPVC) as well as pulmonary stenosis, like you said. Many previous reports encouraged single ventricle palliation. This was a rather large series that underwent biventricular repair, either as a staged or primary repair. The median age of these patients was 16 months. Eleven required VSD enlargement and a right ventricle to pulmonary artery (RV-PA) conduit was required in 12. The pump times averaged 232 minutes, with a cross-clamp time of 156 minutes. These are long and difficult surgeries. There was 1 hospital death and 1 late death. Totally anomalous pulmonary venous connection was significantly associated with increased risk. I have a few questions.
One, regarding the VSD enlargement, how do you really decide on the need for this, as it wasn't done in all patients, and in our limited experience with an inlet VSD with extension to the outlet septum, we have not had to use enlargement techniques.
Two, could you expand on the rationale for a two-patch or composite LVOT/VSD patch as opposed to the more commonly described comma-shaped patch that is used in this entity?
In summary, I enjoyed the paper. It was clear and concise. Very good results with a rare and difficult subset of patients ... and thanks for sending me the manuscript in advance.
DR DEVANEY: Jim, thank you very much for your kind comments and your questions. The first question regarded how do we decide which patients need to have their VSDs enlarged, and I think really you highlighted the issue. It really depends on how much outlet extension there is. In our patient series, certainly the majority of the patients who needed VSD enlargement, we felt needed VSD enlargement, was because the defect was so remote from the aorta that we felt that we couldn't easily create the baffle without precipitating LV outflow tract obstruction. So I can't say in any more detail how we do that. It is just something that we have gotten more comfortable with as we have done more of these cases. We are confident that if we do the muscle resection that we can create an unobstructed outlet. And so that is why it has become a more frequent technique that we have used, especially more recently. As I mentioned, I think 8 of the last 8 we have used this technique.
Now, your second question involved why do we do two separate VSD patches rather than just one. For the patients with tetralogy of Fallot with complete atrioventricular septal defect, I think it is easy enough to see the entire VSD transatrially and to cut the patch accurately. For this lesion, I can't see it well enough. I think it is difficult to see the aortic valve transatrially, and that is why we use a two-patch approach. So this is just a technical feature that makes it easier to achieve consistent good results.
DR CHARLES D. FRASER (Houston, TX): That is a wonderful series and a testimony to excellent technical surgery. However, 2 of your 16 patients died, and I would predict that over that same time period in your institution, the survival for Fontan was virtually 100%. So is this really better than a Fontan? And in an individual patient, how would you justify a more high risk approach like this as opposed to a Fontan operation?
DR DEVANEY: Well, I think you have to look at the denominator and decide. We are not just comparing it to Fontans. We are looking at Fontans in heterotaxy patients, especially those with total anomalous venous connections. I think the risks are higher for that patient group. I think that turns out to be a risk factor for almost any analysis we do, whether it is a single ventricle or a biventricular approach to any surgical lesion. The presence of heterotaxy syndrome and total veins tends to cloud the issue. The deaths and complications are occurring in those patients. So I think we will have tough results no matter which approach we go with. But I think your question is fair, and I really think we need to follow these patients longer term to see whether it is really a justifiable strategy.
DR HARALD L. LINDBERG (Oslo, Norway): Very nice paper, nice results. I just wondered, were there any patients with Down syndrome in this series? Probably not.
DR DEVANEY: There were not.
DR LINDBERG: What was your technique on the zone of opposition or the cleft? Was that closed in all patients?
DR DEVANEY: The zone of opposition was closed, correct.
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Abstract
Introduction
