Data: Among 139 patients who underwent repair of tetralogy with pulmonary atresia, survival rates at 1 month and at 1, 5, 10, and 20 years were 85%, 82%, 76%, 69%, and 58%, respectively. The hazard function (instantaneous risk of dying) was greatest immediately after operation and declined thereafter, but a low constant hazard persisted for as long as the patients were followed up. Multivariately, the postrepair ratio between peak right ventricular and left ventricular pressures measured in the operating room provided the most information relative to the probability of death after repair, and cardiopulmonary bypass time the next. When morphologic abnormalities of the pulmonary circulation were considered in the multivariate analysis for risk factors for death, the size of the pulmonary arteries provided the most information, followed by the number of large aortopulmonary collateral arteries. The postrepair peak right ventricular/left ventricular pressure ratio was lower the day after operation than in the operating room in 65% of the patients in whom the measurements were made. Recurrent or residual ventricular septal defects necessitating rerepair occurred in four patients (3% of hospital survivors). Most surviving patients were in New York Heart Association class I at the time of follow-up.
Inferences: Early, intermediate, and long-term survival is less good after repair of tetralogy with pulmonary atresia than after repair of tetralogy with pulmonary stenosis. This is related primarily to the greater prevalence of high peak right ventricular/left ventricular pressure ratio measured in the operating room in the former group. Both the postrepair peak right ventricular/left ventricular pressure ratio in the operating room and the probability of death are inversely related to the size of the pulmonary arteries and directly to the number of large aortopulmonary collateral arteries. This and inferences from other risk factors may be helpful in achieving better results in the future.