Anatomical and functional hypoplastic left 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 conserving palliation includes not only the Norwood operation during the newborn period but also the complete separation of both circuits by the Fontan operation some time later. Our experiences with each surgical step are presented. From 1989 to 1996, 43 infants with anatomical (n = 33) or functional (n = 10) HLHS underwent the Norwood operation. Functional HLHS were: Mitralatresia with double outlet right ventricle and subaortic stenosis (n = 2), atrioventricular septal defect with hypoplastic 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 operation 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 (3/19 patients). Five infants (12%) died 2 weeks to 6 months later, and 2 patients underwent cardiac transplantation. Up to now, 19 out of the 22 long term survivors underwent the bidirectional cavopulmonary anastomosis (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 received the total cavopulmonary anastomosis after a mean period of 12 months. All children survived, and they are now completely palliated. The longest follow up after the complete Fontan operation is 6 years. Conclusion: With increasing experience the results of the Norwood operation improved. The following two-stage Fontan procedure bears only a low risk and leads to good quality of life.