• F X Schmid, C Kampmann, A A Peivandi, and H Oelert.
• Klinik und Poliklinik für Herz-, Thorax- und Gefässchirurgie, Johannes-Gutenberg-Universität Mainz. schmidfx@mail.uni-mainz.de
• Herz. 1999 Jun 1; 24 (4): 307-14.

AbstractHypoplastic left heart syndrome (HLHS) represents an anatomical spectrum of congenital disease with varying degrees of underdevelopment of the left-sided cardiac structures (Figure 1). The outlook for children born with HLHS, an otherwise highly lethal malformation, has improved with increasing experience with reconstructive techniques. This report represents a detailed analysis of the overall risk and mid-term results for a group of 39 consecutive neonates with HLHS referred to our hospital over a 5-year period between January 1994 and November 1998. Twenty-six patients were treated with a Norwood reconstructive procedure (Figure 2). One patient received a cardiac transplant at the request of the parents. Another patient with aortic atresia, ventricular septal defect and normal left ventricle underwent biventricular repair. Eleven patients were not eligible for surgical treatment due to a number of reasons (Table 1). In 26 neonates (9 girls, 17 boys; mean age 9.1 [4 to 42] days) staged reconstruction by Norwood's procedure was performed. The hospital mortality in the first stage of the Norwood procedure was 23% (6/26). Sixteen of the 20 long-term survivors underwent the bidirectional cavopulmonary anastomosis (hemi-Fontan; Figure 3) at a median age of 7.6 (3 to 14) months. All children survived. Up to now, 3 infants received the complete Fontan operation (Figure 4) at 2 years of age. No late death occurred. In 18 out of the 20 survivors neuro-developmental outcome and exercise performance were within the range of normals. Staged surgical palliation represents a realistic therapeutic option for neonates born with HLHS. At this intermediate stage of follow-up exercise performance and quality of life are satisfactory.

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