Purpose: Hypoplastic Left Heart Syndrome (HLHS) accounts for 7-9% of all congenital heart diseases. It is fatal unless surgically corrected. Survival has improved overtime. However, neurodevelopmental outcomes have not been known until recently. This paper discusses the neurodevelopmental outcomes of patients with HLHS and the factors that influence these outcomes. Methods: A search of the literature was performed using several databases, medical textbooks and other on-line sources to locate research related to HLHS and neurodevelopment in HLHS. Results: Mean performance IQ scores tended to be lower than mean verbal IQ scores. Mean motor scores were below normal population values indicating impairment. Growth was impaired in many of those with HLHS. Factors that had negative impact on outcomes included deep hypothermic circulatory arrest (DHCA), cardiopulmonary bypass (CPB), longer hospital length-of-stay, seizures, and lower socioeconomic status (SES). Conclusions: The studies show that children with HLHS do have neurodevelopmental deficits. Intelligence, motor development and growth are all affected. Focus during care of these patients should be spent on improving the factors discussed that have been shown to have negative impacts on outcomes.



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