Neurodevelopmental outcomes in hypoplastic left heart syndrome

Infants with hypoplastic left heart syndrome (HLHS) are now surviving to school age and early adulthood because of surgical palliation with either staged reconstruction or transplantation. Although in some respects these patients are doing remarkably well, recent investigations have shown that they are at risk for cognitive, neuromotor, and psychosocial problems. This article examines the neurodevelopmental outcome for children and adolescents with HLHS. In addition, hemodynamic and genetic factors unique to this patient population that may impact longer-term outcome are explored. The continued evolution of the pre-, intra- and post-operative care of the patient with HLHS has significantly improved early survival. Concomitant changes in care, such as selective cerebral perfusion and neurologic monitoring, aimed at reducing neurologic insult may also favorably impact later neurodevelopmental status.