Neurodevelopmental outcome and lifestyle assessment in school-aged and adolescent children with hypoplastic left heart syndrome

Objectives. The purposes of this study are to describe the quality of life and cognitive function in school-aged children who have undergone staged pa lliation for hypoplastic left heart syndrome (HLHS), and to identify factor s that are predictive of neurodevelopmental outcome in this population. Methods. School-aged survivors with HLHS who had undergone palliative surge ry at our institution were identified and mailed a questionnaire to assess subjectively quality of life, school performance, and incidence of medical complications. A subgroup of local patients underwent standardized testing of cognitive function and neurologic examination. These patients were compa red with the larger (remote) group of questionnaire respondents to determin e whether results may be generalizable to the entire HLHS population. Poten tial predictors of neurologic and cognitive outcome were tested for their a ssociation with test scores using multivariate regression analysis. Results. Questionnaire results were obtained from 115 of 138 eligible child ren (83%; mean age: 9.0 +/- 2.0 years). Standardized testing was performed in 28 of 34 (82%) eligible local patients (mean age: 8.6 +/- 2.1 years). Th e majority of parents or guardians described their child's health as good ( 34%) or excellent (45%) and their academic performance as average (42%) or above average (42%). One third of the children, however, were receiving som e form of special education. Chronic medication usage was common (64%); the incidence of medical complications was comparable to that previously repor ted in children with Fontan physiology. Cognitive testing of the local grou p demonstrated a median full scale IQ of 86 (range: 50-116). Mental retarda tion (IQ: <70) was noted in 18% of patients. In multivariate analysis, only the occurrence of preoperative seizures predicted lower full scale IQ. Conclusions. Although the majority of school-aged children with HLHS had IQ scores within the normal range, mean performance for this historical cohor t of survivors was lower than that in the general population.