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Ann Thorac Surg 2001;71:49-53
© 2001 The Society of Thoracic Surgeons

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Original article: cardiovascular

Surgical outcome of double-outlet right ventricle with subpulmonary VSD

^a Department of Cardiac Surgery, The Children’s Hospital, Boston, Massachusetts, USA
^b Department of Anesthesiology, The Children’s Hospital, Boston, Massachusetts, USA
^c Department of Cardiology, The Children’s Hospital, Boston, Massachusetts, USA
^d Department of Biostatistics, The Children’s Hospital, Boston, Massachusetts, USA

Address reprint requests to Dr del Nido, Department of Cardiac Surgery, The Children’s Hospital, 300 Longwood Ave, Bader 279, Boston, MA 02115
e-mail: delnido{at}al.tch.harvard.edu

Presented at the Thirty-sixth Annual Meeting of The Society of Thoracic Surgeons, Fort Lauderdale, FL, Jan 31–Feb 2, 2000.

	Abstract

Top Abstract Introduction Material and methods Results Comment Discussion References

 
Background. Optimal management of double-outlet right ventricle with subpulmonary ventricular septal defect remains controversial. We reviewed our 7-year experience with patients who had this anatomic configuration.

Methods. Between January 1992 and January 1999, 20 patients underwent an arterial switch operation (ASO group), and 12 underwent a bidirectional Glenn procedure followed by a modified Fontan in 10 (Glenn/Fontan). Mean follow-up was 23 ± 18 months.

Results. An initial palliative operation was done in 19 patients (9 in the ASO group, 10 in the Glenn/Fontan group). There were no deaths in the Glenn/Fontan group. Four patients in the ASO group died within 33 days postoperatively. Two of them had a single coronary artery, 1 had a straddling mitral valve, 1 had a hypoplastic aortic arch, and 1 had multiple ventricular septal defects. Three patients had reoperation for subaortic stenosis (n = 2) or pulmonary stenosis (n = 1) after the ASO. Four patients (3 in the ASO group, 1 in the Glenn/Fontan) required a pacemaker for postoperative complete atrioventricular block. Actuarial survival at 5 years for the entire group was 87% (70% confidence interval, 81% to 93%).

Conclusions. The ASO remains our preferred treatment for infants with double-outlet right ventricle and subpulmonary ventricular septal defect. However, associated anatomic defects are important risk factors.

	Introduction

Top Abstract Introduction Material and methods Results Comment Discussion References

 
By describing a ventriculoarterial connection rather than a specific congenital malformation, double-outlet right ventricle (DORV) includes a broad spectrum of anatomic variants and associated malformations. Numerous techniques have been presented for anatomic biventricular repair of DORV with a subpulmonary ventricular septal defect (VSD) [1–3], but the optimal management remains controversial. Fontan-type procedures have been advocated when biventricular repair has been either impractical or extremely complex [3, 4]. The arterial switch operation (ASO) with baffling of the VSD to the neo-aorta has become the procedure of choice for the treatment of infants with both DORV and subpulmonary VSD. Potential risk factors include a hypoplastic ventricle (right or left) or atrioventricular (AV) valve, a straddling AV valve, pulmonary stenosis, and side-by-side great arteries with the associated unusual coronary artery pattern. To determine our current results in patients with DORV and subpulmonary VSD, we reviewed our 7-year experience with patients having the ASO or a single-ventricle approach.

	Material and methods

Top Abstract Introduction Material and methods Results Comment Discussion References

 
Between January 1992 and January 1999, 34 patients underwent surgical repair of DORV with a subpulmonary VSD at The Children’s Hospital, Boston. The diagnosis of DORV was made if both great arteries originated preponderantly from the right ventricle with application of the "50% rule" which requires that one great artery arise completely and the second, more than 50% from the right ventricle. The diagnosis and anatomic findings were based on a combination of two-dimensional echocardiographic and angiographic evidence and surgical inspection. Twenty patients underwent the ASO (ASO group) and 12, a bidirectional cavopulmonary connection (Glenn procedure) followed by a total cavopulmonary connection or a modified Fontan procedure in 10 of the 12 (Glenn/Fontan group). Two patients underwent a Rastelli-type repair or a Damus-Kaye-Stansel procedure. The indications for a single-ventricle approach were based on complex anatomy such as a straddling AV valve not amenable to repair, hypoplasia of the right or left ventricle, or difficult coronary artery anatomy for an ASO (surgeon’s preference). Age at operation ranged from 1 day to 53 months (mean age, 13.4 months; median age, 6 months).

Patient data were compiled by review of the clinical records (including operative reports) and the preoperative angiographic and two-dimensional echocardiographic studies. Time to follow-up ranged from 1 day to 5.6 years, with a mean follow-up of 23 ± 18 months (median follow-up, 1.1 years).

Potential risk factors for early mortality were analyzed in contingency tables with ² test or Fisher’s exact test. Multivariate analyses of early survival and reoperation-free survival were performed with stepwise logistic regression and Cox regression methods. Survival estimates were made using the Kaplan-Meier method. All analyses were performed with the use of the standard commercially available software package SPSS (SPSS Inc, Chicago, IL).

	Results

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Nineteen patients, 9 in the ASO group and 10 in the Glenn/Fontan group, had undergone previous palliative procedures before the ASO or Glenn procedure (Table 1). Associated anatomic lesions are listed in Table 2. A side-by-side relationship between the aorta and the pulmonary artery or unusual coronary artery pattern was seen in 10 patients in the ASO group and in 6 in the Glenn/Fontan group.

Three patients required late reoperation for subaortic stenosis (n = 2) or pulmonary stenosis (n = 1) after the ASO (mean period to reoperation, 19 months). Four patients, 3 in the ASO group and 1 in the Glenn/Fontan group, required permanent pacemaker insertion for postoperative complete AV block.

Overall survival according to Kaplan-Meier analysis at 1 month, 1 year, and 4 years was 90.6% (70% confidence interval [CI], 85.5% to 95.5), 87.3% (70% CI, 81.3% to 93.3%), and 87.3% (70% CI, 81.3% to 93.3%) respectively. Survival by type of operation is shown in Figure 1. Logistic regression (non-time-related analysis) revealed that the odds of death were significantly higher for the ASO group than the Glenn/Fontan group (p = 0.04). Cox multivariate regression analysis revealed that the only significant independent predictor of mortality was side-by-side great arteries. Estimated 1-year survival (univariate analysis by the Kaplan-Meier method) was significantly worse for patients with side-by-side great arteries (72% [70% CI, 58% to 86%]) compared with patients with other great-artery relationships (95% [70% CI, 90% to 100%]) (p = 0.05, log-rank test).

View larger version (16K): [in this window] [in a new window]   Fig 1. Kaplan-Meier survival curve by type of operation for children undergoing surgical repair of double-outlet right ventricle with subpulmonary ventricular septal defect. (ASO = arterial switch operation; BDG = bidirectional Glenn procedure.)

	Comment

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Double-outlet right ventricle includes a wide spectrum of anatomic subtypes requiring a variety of surgical approaches for correction. Double-outlet right ventricle with subpulmonary VSD, including the Taussig-Bing anomaly, which is characterized by side-by-side semilunar valves and a bilateral conus, has been considered a complex form of DORV. The complexity of the repair is due mainly to the frequency of associated anomalies that can affect the ability to achieve a two-ventricle repair without the use of an extraanatomic conduit [1].

Numerous techniques have been described for biventricular repair of DORV with a subpulmonary VSD: the ASO, intracardiac baffling including the Rastelli operation or a variant described by Kawashima and associates [1], and the Damus-Kaye-Stansel procedure [2, 3]. The ASO, which was introduced as a corrective technique for simple transposition of the great arteries, has become the procedure of choice for patients with all forms of dextrotransposition including those with DORV. At our institution, the ASO is currently the most commonly used technique for two-ventricle repair of DORV with a subpulmonary VSD. As experience has been gained with management of both complex coronary artery anatomy and associated aortic arch anomalies, the ASO has been more widely used in the subgroup of patients with transposition and DORV [5].

In this study we found the probability of death in the ASO group to be 20%, which is higher than that described in a previous report from our group [6] concerning the surgical outcome of all patients with DORV. However, in that series, only 33% of the children with DORV and subpulmonary VSD had an ASO compared with 62.5% in this study, thus indicating a more selective approach in the previous series. On the basis of our more recent results [5] indicating that coronary anatomy is not a risk factor for death when analyzing results of all infants undergoing the ASO, we have slanted our surgical management of DORV with a subpulmonary VSD more toward an anatomic two-ventricle repair, even in more complex cases. In that review, however, we also found that the Taussig-Bing type of DORV per se and side-by-side great arteries were risk factors for the ASO. Mavroudis and associates [7] reported good medium-term results of the ASO in a select group of patients and good results in a small cohort (4 patients) who underwent a Kawashima type of intraventricular repair with an extracardiac conduit for patients with Taussig-Bing anomaly. However, None of the children undergoing the Kawashima procedure had straddling AV valves.

In our review, side-by-side great vessel anatomy was identified as an independent risk factor by Cox multivariate regression analysis. Two of the deaths were directly related to problems with coronary artery translocation in infants with an unusual coronary artery pattern and side-by-side great arteries. The high association between side-by-side great vessels and unusual coronary artery pattern has been emphasized previously. In a detailed pathologic study, Uemura and associates [8] found that a single coronary artery was present in 27% of hearts with a side-by-side great artery relationship. Gordillo and colleagues [9] also described a higher incidence of unusual coronary artery pattern in DORV with a subpulmonary VSD. The higher risk associated with side-by-side great arteries may also be related to the higher incidence of aortic arch anomalies and deviation of the outlet septum toward the aorta [10].

The modified Fontan operation [11] has been widely applied as the surgical procedure of choice in the presence of complex anatomic features such as hypoplastic AV valves or ventricles. These two anatomic features along with a straddling AV valve were the indications for a single-ventricle type of management in the patients in this series. Although single-ventricle management is less desirable because of the potential late complications of arrhythmia, decreased exercise tolerance, and protein-losing enteropathy [11], surgical modifications have had a favorable impact on outcome [12, 13]. Staged procedures (Glenn procedure followed by Fontan operation) and a lateral tunnel with a fenestration or an extracardiac conduit have resulted in considerable improvement in the short- and medium-term results for the modified Fontan operation [14, 15]. In the present series, there was no hospital mortality among patients receiving the modified Fontan procedure as the definitive surgical management. The difference in early mortality between patients with complex anatomic features undergoing biventricular repair and those having some form of Fontan procedure suggests that the latter may be the procedure of choice for this subgroup. However, risk factors such as coronary artery pattern and the presence of side-by-side great vessels may be neutralized with increased surgical experience [5].

In conclusion, the independent risk factor for the ASO in patients with DORV and subpulmonary VSD was the presence of side-by-side great arteries, which is likely related to the strong association with coronary artery anomalies, aortic arch obstruction, or both. Single-ventricle management of DORV, subpulmonary VSD, and associated complex anatomic defects is an acceptable alternative with excellent early and midterm results. The low operative risk and encouraging intermediate results should be weighed against the potential long-term complications of the Fontan operation. Currently our preference is to perform an ASO in all patients with DORV and subpulmonary VSD unless hypoplasia of a ventricle or an AV valve or a straddling AV valve is present.

	Discussion

Top Abstract Introduction Material and methods Results Comment Discussion References

 
DR KIRK R. KANTER (Atlanta, GA): You mentioned mitral valve abnormalities, specifically, a straddling mitral valve.

DR TAKEUCHI: One patient who died had a straddling mitral valve. This may have been related to the death, but we cannot say that this feature has significance.

DR CHRISTOPHER J. KNOTT-CRAIG (Oklahoma City, OK): I enjoyed your presentation very much and congratulate you and the team from Boston on excellent results in this very difficult group of patients.

I will play devil’s advocate and ask you to comment on one procedure that carries a 20% mortality rate and a 20% reintervention rate at 2 years compared with another procedure that carries a 0% mortality rate and a fairly good outcome at 3 years. What role do you think the arterial switch operation has in patients with the Taussig-Bing anomaly? Should we be converting more of these patients to single-ventricle repair?

DR TAKEUCHI: Thank you for your questions. In total, 4 patients died in this series. One patient died of cerebral hemorrhage and can be excluded from this discussion. Two patients died of myocardial ischemia, and this may have been related to the translocation of the coronary artery to the neo-aorta. The fourth patient died of severe right ventricular failure with respiratory failure. Right ventricular failure can be related to multiple ventricular septal defects, and this patient had multiple VSDs.

Our strategy is aimed toward the two-ventricle repair because it is more physiologic. In the last series, we [6] also evaluated patients with double-outlet right ventricle. Seventy-three patients had two-ventricle repair, and 27 patients had a subpulmonary VSD. In that series, only 33% of patients had an arterial switch operation compared with more than 60% of patients in this series. What we tried to do is to lean toward the two-ventricle repair. However, it is important to be careful with the coronary anatomy.

DR CHRISTO I. TCHERVENKOV (Montreal, Canada): I congratulate you on a nice presentation. My discussion and comments focus on two high-risk groups, the one with aortic arch obstruction and the one with subaortic stenosis.

At the Montreal Children’s Hospital, my colleagues and I have adopted a single-stage approach for the surgical repair of patients with the Taussig-Bing heart or transposition complexes with aortic arch obstruction. The hypoplastic aortic arch is repaired at the same time the arterial switch operation and the VSD closure are done. Our technique involves pulmonary homograft patch aortoplasty through a sternotomy to augment the entire hypoplastic aortic arch.

With this technique, we achieve a number of things. First is the avoidance of two circumferential suture lines, one at the neo-aortic anastomosis and the other in the aortic arch, that can be associated with excessive tension. Second, this technique allows for the enlargement of the frequently observed hypoplastic distal ascending aorta and the correction of the marked size discrepancy between the proximal and distal ends of the aorta.

In our experience with 20 patients undergoing a single-stage arterial switch operation with concomitant aortic arch repair, we have had no perioperative mortality. Nine of those 20 patients had the Taussig-Bing malformation. I am interested to know how many of your patients received the single-stage repair. What was your technique for enlarging the aortic arch? The second high-risk group is the one with severe subaortic stenosis, which is often associated with aortic arch obstruction. We have performed a two-ventricle repair in the neonate with severe subaortic obstruction and a coronary artery crossing the right ventricular outflow tract precluding a transannular incision. We have used the ventricular septal defect as a systemic outflow from the left ventricle into the proximal main pulmonary artery, which, when transected, is connected to the systemic circulation. The two-ventricle repair is completed by the establishment of right ventricle–distal pulmonary artery continuity with a valved pulmonary homograft. Have you used or considered this technique of two-ventricle repair in any of your patients?

What were the factors that precluded a two-ventricle repair in the patients who underwent a single-ventricle approach?

DR TAKEUCHI: Thank you for your comments. In this series we did see hypoplastic arch, in 5 patients. Because most of the aortic arch problems were coarctation, we did coarctation repair before the definitive operation. However, that was early in the series, in 1992 through 1994. Now we try to perform the single-stage operation even if coarctation is present.

In a different series, I saw the type of operation you refer to, that is, augmenting the hypoplastic aortic arch with a homograft. I agree that we can enlarge the aortic arch and do the switch operation simultaneously. I think the arterial switch operation can be more widely applied in many situations.

Regarding your second question, mild to moderate subaortic stenosis was not an indication for single-ventricle management, and we can apply several techniques in such patients.

	References

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