ANATOMICAL AND FUNCTIONAL HYPOPLASTIC LEF T-HEART SYNDROME AND ITS SURGICAL THERAPY BY NORWOOD AND FONTAN OPERATION

The surgical therapy of newborns with hypoplastic left heart syndrome (HLHS) is still regarded with some distrust. The complete heart conser ving palliation includes not only the Norwood operation during the new born period but also the complete separation of both circuits by the F ontan operation some time later. Our experiences with each surgical st ep are presented. From 1989 to 1996, 43 infants with anatomical (n = 3 3) or functional (n = 10) HLHS underwent the Norwood operation. Functi onal HLHS were: Mitralatresia with double outlet right ventricle and s ubaortic stenosis (n = 2), atrioventricular septal defect with hypopla stic left ventricle, subaortic stenosis, and aortic coarctation (n = I ), hypoplastic, subaortic right ventricle with restrictive ventricular septal defect and aortic hypoplasia (n = 7). The median age at operat ion was 15 days (5 to 182 days), mean weight was 3.3 kg (3.0 to 4.9 kg ). Total operative mortality was 32% (n = 14) with 16% since 1994 (3/1 9 patients). Five infants (12%) died 2 weeks to 6 months later, and 2 patients underwent cardiac transplantation. Up to now, 19 out of the 2 2 long term survivors underwent the bidirectional cavopulmonary anasto mosis (Hemi-Fontan) at a median age of 7 months (2 to 14 months). Two infants died (10%). Up to now, 12 out of the remaining 17 survivors re ceived the total cavopulmonary anastomosis after a mean period of 12 m onths. All children survived, and they are now completely palliated. T he longest follow up after the complete Fontan operation is 6 years. C onclusion: With increasing experience the results of the Norwood opera tion improved. The following two-stage Fontan procedure bears only a l ow risk and leads to good quality of life.