Pulmonary atresia: surgical considerations and results in 103 patients undergoing definitive repair

Surgical considerations and results of repair of 103 patients with pulmonary atresia are reviewed. The operative mortality was 10%, related primarily to inability to relieve right ventricular hypertension and to low cardiac output after complex repairs involving significant associated anomalies. In a follow-up study ranging from 6 months to 8 years, there were four late deaths, each being a result of persistent right ventricular hypertension. All but one of the surviving patients are in Class I or Class II. Since operative and late mortality and morbidity are related to unrelieved right ventricular hypertension due to restricted pulmonary arterial outflow, it should be possible to improve results by performance of a preliminary systemic-pulmonary arterial shunt for patients with hypoplastic pulmonary arteries and by correction of patients with large systemic-pulmonary arterial shunts before the development of pulmonary vascular obstructive disease. The striking relief of cyanosis, exercise intolerance, and other symptoms, and the generally favorable operative risk, justify the continued application of surgical correction of pulmonary atresia.