Surgical treatment of hypoplastic left heart syndrome: Experience with staged palliative reconstruction

Hypoplastic left heart syndrome (HLHS) represents an anatomical spectrum of congenital disease with varying degrees of underdevelopment of the left-si ded cardiac structures (Figure 1). The outlook for children born with HLHS, an otherwise highly lethal malformation, has improved with increasing expe rience with reconstructive techniques. This report represents a detailed an alysis of the overall risk and mid-term results for a group of 39 consecuti ve 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 (F igure 2). One patient received a cardiac transplant at the request of the p arents. 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). I n 26 neonates (9 girls, 17 boys; mean age 9.1 [4 to 42] days) staged recons truction 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 c avopulmonary 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 com plete Fontan operation (Figure 4) at 2 years of age. No late death occurred . In 18 out of the 20 survivors neurodevelopmental outcome and exercise per formance were within the range of normals. Staged surgical palliation repre sents a realistic therapeutic option for neonates born with HLHS. At this i ntermediate stage of follow-up exercise performance and quality of life are satisfactory.