Results of modified Norwood's operation for hypoplastic left heart syndrome.

ABSTRACT

OBJECTIVE: The aim of the study was to analyze intermediate results of treatment of the hypoplastic left heart syndrome based on selective indication criteria. METHODS: Between February 1997 and May 1999 38 patients with hypoplastic left heart syndrome (n=35), or with functional variant of hypoplastic left heart syndrome (n=3) were admitted to our department. Contraindications for surgery were birth weight <2500 g, diameter of ascending aorta <2 mm, severe tricuspid regurgitation persisting after initial stabilization, pulmonary regurgitation more than mild, dysfunction of the systemic right ventricle and failure to effectively resuscitate circulation before surgery. RESULTS: Based on these criteria surgery was not indicated in 17 patients. Twenty-one infants were operated on by modified Norwood's procedure using only autologous great vessel tissue for reconstruction of systemic outflow. Overall hospital mortality was 14% (three patients). Eighteen survivors (86%) were discharged with well-balanced circulation. There was one late death (5%). Thirteen patients had already undergone the second stage (bi-directional Glenn) with no death. The mean follow-up was 13. 2+/-9.1 months (range 4-32 months). Considering both early and late events the probability of survival for the whole group (n=21) from the time of surgery was 86% at 1 month, 80% at 12 months, and it remained unchanged at 18 and 24 months of follow-up. CONCLUSIONS: Only a limited number of European countries offer surgical treatment of hypoplastic left heart syndrome. Promising intermediate results (80% survival rate after stage I and II) achieved at our department do not only reflect overcoming the learning curve but also a selective approach to indication for surgery as well. In a country with limited resources selective approach to the patients with hypoplastic left heart syndrome is justified.