Neurodevelopmental Outcomes in Patients with Hypoplastic Left Heart Syndrome

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. INTRODUCTION Hypoplastic Left Heart Syndrome (HLHS) is a congenital condition that involves a spectrum of cardiac malformations characterized by underdevelopment of the left heart structures.1,2,3 HLHS is a lethal cardiac abnormality if not surgically corrected.3 Over time, survival rates in these patients have improved. However, neurodevelopmental outcomes in these patients have not been known until recently. This paper will describe the complex syndrome of HLHS and the surgical management of it and will focus on the neurodevelopmental outcomes of these patients. The specific outcomes used to measure neurodevelopmental status and the factors that influence these outcomes will be discussed. METHODS A thorough search of English language literature was conducted using Medline, EMBASE, ERIC, and PsycInfo databases on all research related to HLHS. All of the research articles that looked at neurodevelopment in patients with HLHS were included. Subjects of all ages were included. No time frame for the search was pre-specified. However, the papers identified and reviewed ranged from 1995 through 2008. All surgical techniques used on these patients for the treatment of HLHS were included. A thorough search was also conducted in Cochrane and other databases for systematic reviews and meta-analyses on this topic. No such papers were found that provided neurodevelopmental data specifically on HLHS patients. Medical textbooks and online, continually-updated, reliable medical resources were also used for supportive data.