[Early and late results after surgical treatment of pulmonary atresia with intact ventricular septum].

Between 1970 und 1989 30 children were admitted with the diagnosis of pulmonary atresia with intact ventricular septum (PA/IVS). Before palliation 4 children died. According to the grade of right heart hypoplasia the patients were divided into 3 groups of mild, moderate or severe hypoplasia. Palliative operations were performed in 25 children (17 m, 9 f) with a mean age of 10 days: 13 valvotomies (V), 5 aortopulmonary shunts (S), 7 V plus S. 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 14/30 (54%) children: early 10/26 (38%), late 4/16 (25%) children. After total correction mortality was 3/7 (43%) children. Actuarial survival after palliation was 46% after 5 and 10 years. For patients with PA/IVS we recommend the following surgical strategy: 1. mild hypoplasia: V plus S for palliation; 2. moderate hypoplasia: S plus patch of RVOT; 3. severe hypoplasia: after initial ballon septostomy S and antegrade decompression of the right ventricle (RV). For total correction in a well developed RV we prefer ASD-closure and patch of RVOT if possible with homograft monocusp. In moderate or severe hypoplasia a Fontan operation is done with closure of the ASD and tricuspid orifice with a single patch.