Staged repair of pulmonary atresia with ventricular septal defect and major systemic to pulmonary artery collaterals.

Fifty-eight consecutive patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries with arborization defects, and major aortopulmonary collaterals were entered into a program for staged surgical repair between January 1979 and July 1989. Prerepair preparatory procedures were designed to (1) encourage native pulmonary artery growth by increasing blood flow and (2) unifocalize pulmonary blood supply by transplanting or ligating major collaterals. A total of 121 staging procedures were performed with an overall mortality of 10.3%. One hundred thirty-four major collaterals were either ligated or transplanted. Thirty patients eventually underwent hemodynamic repair with an early mortality of 3.3% and late mortality of 10.0%. Twenty-six current survivors of repair remain clinically well after a mean follow-up of 3.6 years. Ten patients are currently in various stages of preparation. Twelve patients (20.7%) failed to achieve minimum requirements for repair after staging and await further palliation or heart-lung transplantation. The principles of management have evolved over the years and are discussed.