Analysis of survival in patients with pulmonary atresia and intact ventricular septum treated surgically.

The epidemiology of 98 children who underwent surgical intervention for pulmonary atresia and intact ventricular septum is presented. Sixty-one (62.2%) of the children died. Survival time from the date of the first operation ranged from 0 to 17.76 years. Analysis of the survivors revealed that an estimate of the probability of surviving for two years is 47% (95% confidence limits of 37%, 57%). An estimate of the median survival time is 1.43 years. In a Cox proportional hazards model, using survival time in days as the outcome variable (n = 73, using complete data), operative weight at first operation (P = 0.0019), right/left ventricular pressure ratio (P = 0.0185), and absence of ventriculo-coronary arterial connections (P = 0.0362) were identified as significant predictors of survival.