Unifocalization of major aortopulmonary collateral arteries in pulmonary atresia with ventricular septal defect is essential to achieve excellent outcomes irrespective of native pulmonary artery morphology.

OBJECTIVE: Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is a complex lesion with a high rate of natural attrition. We evaluated the outcomes of our strategy of unifocalization in the management of these patients.

METHODS: From 1989 to 2008, 216 patients entered a pathway aiming for complete repair by unifocalizing major aortopulmonary arteries to a right ventricle-pulmonary artery conduit with ventricular septal defect closure. Where ventricular septation was not possible, definitive repair was considered to include pulmonary artery reconstruction and a right ventricle-pulmonary artery conduit or systemic shunt. Native pulmonary artery morphology was classified into confluent intrapericardial (n = 139), confluent intrapulmonary (n = 51), and nonconfluent intrapulmonary (n = 26).

RESULTS: A total of 203 patients (85%) had definitive repair at a median age of 2.0 years. There was no statistically significant difference in survival after complete repair among the 3 morphologic pulmonary artery groups (P = .18). A total of 132 patients (56%) had complete repair with ventricular septal defect closure, as a single procedure in 111 patients and a staged procedure in 21 patients. Focalization of major aortopulmonary collateral arteries with proven long-term patency with the right ventricle was associated with a survival benefit compared with 14 patients in whom unifocalization was not possible and who had only systemic shunts. Overall survival was 89% at 3 years after definitive repair. During follow-up, 190 patients required 196 catheter reinterventions and 60 surgical reinterventions.

CONCLUSION: By using a strategy of unifocalization, intrapericardial pulmonary artery reconstruction, and right ventricle-pulmonary artery conduit, excellent long-term survival can be achieved in this group of patients even in the absence of native intrapericardial pulmonary arteries.