Objective: We report our experience with repair of tetralogy of Fallot associated with complete atrioventricular septal defect, addressing in particular the need for a pulmonary valve in the right ventricular outflow tract.
Methods: Between 1992 and 2006, 33 children with tetralogy of Fallot and complete atrioventricular septal defect were admitted; 26 had Down's syndrome (79%). Thirty-two children had complete repair (18 primary, 14 staged); of the 15 who received initial palliation, 1 died before complete repair. Right ventricular outflow tract obstruction was relieved by transannular patch in 14 cases (42%), infundibular patch with preservation of the pulmonary valve in 7 (21%), and right ventricle-to-pulmonary artery conduit in 11 (33%).
Results: There were no hospital deaths. Actuarial survival was 96% +/- 3.9% at 5 years and 85.9 +/- 1.1% at 10 years. Multivariate analysis showed that type of relief of right ventricular outflow tract obstruction did not influence survival (P = .16), nor did the choice to use a valved conduit (P = .82). Primary correction (P = .05) and lower weight at repair (P = .05) were associated with higher probability of survival. Mean follow-up was 69.3 +/- 5.9 months (range 0.2-282 months). There were 2 late deaths. Overall freedom from reoperation was 69% at 5 years and 38% at 10 years. Right ventricular outflow tract reconstruction without use of a valved conduit allowed a significantly higher freedom from reinterventions (P < .05).
Conclusions: Tetralogy of Fallot associated with complete atrioventricular septal defect can be corrected at low risk with favorable intermediate survival. Use of right ventricle-to-pulmonary artery conduit can be avoided in two thirds of patients with no impact on survival, possibly improving overall freedom from reintervention.