title: [Early and late results after surgical treatment of pulmonary atresia with intact ventricular septum].
abstract: 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.
authors.affiliation: Klinik für Herzgefässchirurgie, Universitätsspital Zürich.
authors.forename: U
authors.initials: U
authors.lastname: Niederhäuser
authors.forename: E P
authors.initials: EP
authors.lastname: Bauer
authors.forename: L K
authors.initials: LK
authors.lastname: von Segesser
authors.forename: T
authors.initials: T
authors.lastname: Carrel
authors.forename: A
authors.initials: A
authors.lastname: Laske
authors.forename: M
authors.initials: M
authors.lastname: Schönbeck
authors.forename: U
authors.initials: U
authors.lastname: Arbenz
authors.forename: M
authors.initials: M
authors.lastname: Turina
mesh_terms: D002423:Cause of Death; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery*; D006352:Heart Ventricles / Q000002:abnormalities / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010166:Palliative Care; D011183:Postoperative Complications / Q000401:mortality*; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000601:surgery; D011664:Pulmonary Valve / Q000002:abnormalities* / Q000601:surgery; D012189:Retrospective Studies
vernacular_title: Früh- und Spätresultate nach operativer Behandlung von Pulmonalatresien mit intaktem Ventrikelseptum.