Between 1970 and 1989 26 children were operated for pulmonary atresia with intact ventricular septum (PA/IVS). According to the degree of right heart hypoplasia the patients were divided into 3 groups of mild (5), moderate (17) or severe (4) hypoplasia. Palliative operations were performed in 25 children (17 male, 8 female) at a mean age of 10 days: 13 valvotomies (valv.), 5 aortopulmonary shunts, and 7 valv. plus shunt. One patient had total correction as primary procedure. A total of 17 reoperations was necessary in 12 of 26 patients (10 palliations, 7 total corrections). Total corrections were: 2 conduits and 5 patches of the right-ventricular outflow tract (RVOT). Total mortality was 14/26 (54%) children (early 10/26 = 38%, late 4/26 = 16%). After total correction mortality was 3/7 (43%) patients. After a mean follow up of 10.8 years after palliation the 12 survivors are mostly in NYHA class I. Actuarial survival after palliation was 60% after 30 days and 44% after 5 and 10 years. We analyzed 9 clinical and hemodynamic variables by univariate and multivariate analysis to assess the predictive factors of postoperative outcome. Multivariate analysis disclosed the degree of right-ventricular hypoplasia (p = 0.023) as an independent predictor for death whereas the age at palliation only approached significance (p = 0.065). We recommend the following surgical strategy, aiming in the first place at decompressing the right ventricle: in mild hypoplasia valvotomy alone or combined with a shunt for palliation, in moderate hypoplasia shunt plus patch of the RVOT and in severe hypoplasia shunting alone. In case of a restrictive foramen ovale initial balloon septostomy is performed.(ABSTRACT TRUNCATED AT 250 WORDS)