Published online by Cambridge University Press: 19 August 2008
Three children with pulmonary atresia and ventricular septal defect with absent central pulmonary arteries underwent successful complete correction following preliminary staging procedures for unifocalization and deletion of multiple major aortopulmonary collateral arteries. The unifocalization procedures were accomplished using valveless pulmonary arterial cryopreserved allografts. This technique was found to facilitate and simplify the final repair. All patients survived the complete correction. The patients have been followed for 1½-4 years following the final surgery. One child died two years after repair from pneumonia complicated by asthma. One patient appears to have progression of pulmonary vascular disease despite the complete repair, and one patient continues to do well both symptomatically and hemodynamically. The results suggest that this method of unifocalization solves many of the problems inherent to more classical techniques, but the overall prognosis of patients in this diagnostic entity has yet to be completely defined.