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.