EARLY DEVELOPMENTAL OUTCOME AFTER THE NORWOOD PROCEDURE FOR HYPOPLASTIC LEFT-HEART SYNDROME

Objective. To assess intellect and adaptive behavior in children with hypoplastic left heart syndrome (HLHS) who had undergone at least two surgical stages of the Norwood procedure. Methods. Fourteen children w ith HLHS >3 years of age participated in the study. The patients under went intelligence quotient (IQ) testing, and their parents were interv iewed regarding their children's adaptive behavior. Results were compa red with those of 10 family controls. Outcomes were studied for possib le correlation with perioperative variables. Results. Among the HLHS p atients, the median scores for full scale IQ and adaptive behavior wer e 88 and 91, respectively (normal = 100 +/- 15). One child met criteri a for mental retardation. Family controls scored generally higher than did HLHS patients, but only differences in adaptive behavior were sta tistically significant. A negative correlation was found between stage I circulatory arrest time and full scale IQ. Conclusions. Children wi th HLHS most often function in the low-normal range of intelligence an d adaptive behavior. A prolonged circulatory arrest time may result in decreased intellectual function.