Between 1980 and 1991, 45 patients with pulmonary atresia with intact interventricular septum (38 cases) or critical pulmonary stenosis (7 cases), underwent surgery. The right ventricle was tripartite in 23 children, bipartite in 12 and unipartite in 10. Sinusoid vessels were present in 20 cases. They reinjected a native coronary trunk in 10 cases (major sinusoids). During the initial operation performed in the neonatal period in 41 cases and later in 4 cases, 26 children had an isolated systemic-pulmonary shunt (3 deaths) and 19 had connection of the right ventricle and pulmonary artery (6 deaths). Perioperative mortality was 20% (9/45). Global actuarial survival at 5 years was 47%. Of the 22 patients referred later for univentricular repair, 8 (36%) died (6 perioperative and 2 sudden deaths). The actuarial survival for this group was 49% at 4 years. Of the 23 patients referred for biventricular repair, 11 (47%) died (9 perioperative and 2 sudden deaths) and 2 attained complete cure status. The actuarial survival is 43% at 4 years in this group. Children with intermediate forms (type II or tricuspid less than 8 mm) were orientated to univentricular repair, partly due to the high incidence of major sinusoids. None of the children with major sinusoids survived to over 3 years of age. When the anatomic form is favorable, the present strategy is to perform transpulmonary valvotomy under cardiopulmonary bypass associated with the implantation of a Gore-Tex tube between the innominate artery and right pulmonary artery during the neonatal period. The risk of secondary pulmonary regurgitation would seem to be less than the immediate risk of low pulmonary flow.(ABSTRACT TRUNCATED AT 250 WORDS)