EARLY AND LATE RESULTS AFTER OPERATIVE 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.