Original article
Cardiovascular
Analysis of Surgical Outcome in Complex Double-Outlet Right Ventricle With Heterotaxy Syndrome or Complete Atrioventricular Canal Defect

https://doi.org/10.1016/j.athoracsur.2006.02.007Get rights and content

Background

Double-outlet right ventricle encompasses a broad spectrum of anomalies. Heterotaxy syndrome, which is often associated with total anomalous pulmonary venous connection and complete atrioventricular canal defect, has been considered a risk factor for surgical repair of double-outlet right ventricle.

Methods

From January 1992 to May 1999, medical records of 96 patients (50 males, 46 females) who had complex double-outlet right ventricle with heterotaxy and/or complete atrioventricular canal defect were reviewed (median age at initial surgery 3 months). Seventeen patients were neonates requiring surgery. Follow-up ranged from 1 day to 7.4 years (median, 16 months).

Results

Sixty-eight patients had heterotaxy syndrome (27 with total anomalous pulmonary venous connection). Eighty-three had complete atrioventricular canal defect, 22 with moderate to severe atrioventricular valve regurgitation at the time of surgical repair. Eight patients had two-ventricle repair, and 88 patients were considered for single-ventricle management (bidirectional Glenn, 37; Fontan, 44). One patient had heart transplantation after bidirectional Glenn. There were 16 deaths including 10 early (<30 days postoperatively). Overall survival (95% confidence interval) estimated by the Kaplan–Meier method was 89% (83% to 96%) at 1 month, 84% (76% to 91%) at 1 year, and 81% (73% to 89%) at 5 years. Multivariate analysis revealed that neonatal presentation requiring surgery (p < 0.0001), moderate to severe atrioventricular valve regurgitation (p = 0.03), and pulmonary venous obstruction (p = 0.02) were risk factors for death.

Conclusions

Atrioventricular valve regurgitation, pulmonary venous obstruction, and neonatal presentation are risk factors for mortality in patients with complex double-outlet right ventricle. Early surgical intervention in symptomatic neonates and infants, including those with pulmonary venous obstruction, may reduce mortality and improve outcome after staged operation.

Section snippets

Patients

From January 1992 to May 1999, 96 patients (50 males, 46 females) who had complex DORV with heterotaxy or CAVC defect and were admitted to Children’s Hospital, Boston, Massachusetts, for surgery were eligible for entry into the study. Patients were retrospectively identified by review of the cardiac surgical, echocardiography, and cardiac catheterization laboratory databases. Assignment to the asplenia or polysplenia group was done according to the findings at echocardiography using criteria

Results

Between January 1992 and May 1999, 96 children (50 males, 46 females) with complex DORV (with heterotaxy or CAVC) were admitted to Children’s Hospital, Boston, and underwent surgery. Median follow-up was 16 months (range, 1 day to 7.4 years). As depicted in Table 1, of these 96 children, 68 patients had heterotaxy syndrome, 83 patients had CAVC defect, including 37 patients with unbalanced form of AV canal defect, and 30 patients had TAPVC. Fifty-six patients had both heterotaxy syndrome and

Comment

In this study, we found that AVVR, pulmonary venous obstruction, and neonatal presentation, but not TAPVC, are independent risk factors for mortality in patients with complex DORV with heterotaxy and/or CAVC.

Double-outlet right ventricle encompasses a broad spectrum of anatomic variants and associated malformations. Heterotaxy syndrome, which is often associated with TAPVC and CAVC, has been considered a risk factor for surgical repair of DORV. The early and mid-term outcomes for children with

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