Analysis of surgical outcome in complex double-outlet right ventricle with heterotaxy syndrome or complete atrioventricular canal defect

Ann Thorac Surg. 2006 Jul;82(1):146-52. doi: 10.1016/j.athoracsur.2006.02.007.


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.

Publication types

  • Evaluation Study

MeSH terms

  • Abnormalities, Multiple / surgery
  • Child
  • Child, Preschool
  • Double Outlet Right Ventricle / surgery*
  • Female
  • Follow-Up Studies
  • Heart Defects, Congenital / surgery
  • Heart Septal Defects / surgery
  • Heart Transplantation
  • Humans
  • Infant
  • Infant, Newborn
  • Life Tables
  • Male
  • Mitral Valve Insufficiency / complications
  • Postoperative Complications / mortality
  • Pulmonary Veins / pathology
  • Retrospective Studies
  • Risk Factors
  • Survival Analysis
  • Treatment Outcome
  • Tricuspid Valve Insufficiency / complications