[Mortality and survival rate in pulmonary atresia with intact intraventricular septum. Determining factors].

Sixty nine children with pulmonary atresia and intact ventricular septum diagnosed by catheterization from 1971 to 1988 are reviewed. These patients are the 2.9% of the 2,322 children diagnosed of congenital heart disease by catheterization and angiography in this 17 year period. The overall mortality was 72.4%. Twenty children died at a mean age of 37 days before cardiac surgery was performed, mostly before 1982, before the use of prostaglandin infusion. Forty seven children received surgical treatment, with a surgical mortality rate of 74% at the period 1971-1982 and 54% at the period 1983-1988. The right ventricular hypoplasia was the anatomic factor of dismal outlook. The actuarial survival in the patients without hypoplastic right ventricle and surgical treatment was 52% in the period 1971-1988 and near 80% in the first 3 years of life in the period 1983-1988. The use of preoperative prostaglandin infusion and improved operative techniques has changed the natural history of this malformation.