Surgical management of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a protocol-based approach.

Historically, outcomes of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals have been quite poor. Over the past 15 years, our group has strived to apply a consistent surgical strategy for this lesion based on two guiding principles: early unifocalization of all important collaterals; and the early establishment of a low-pressure pulmonary arterial bed, preferably using simultaneous intracardiac repair. We describe a management protocol that has been developed to handle the highly variable nature of pulmonary blood flow observed with this lesion.