Surgical management of pulmonary atresia with ventricular septal defect and multiple aortopulmonary collaterals

Pulmonary atresia with ventricular septal defect and multiple aortopulmonary collaterals is a form of complex congenital heart disease that is the subject of continued controversy. Disagreement exists regarding the appropriateness of surgical therapy, the timing of operation, and the optimal techniques for repair (1,2). The reason for these differences centers around the diversity of morphologic and consequent physiologic forms with which this defect presents. The relatively small number of patients and large number of subgroups make meaningful comparison between differing treatment strategies difficult. We present here the approach to these patients that is currently employed at UCLA Medical Center.