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PEDIATRICS Vol. 105 No. 5 May 2000, pp. 1082-1089

Neurodevelopmental Outcome and Lifestyle Assessment in School-Aged and Adolescent Children With Hypoplastic Left Heart Syndrome

Received Jan 4, 1999; accepted Jul 8, 1999.

William T. Mahle*, parallel , Robert R. ClancyDagger , parallel , , Edward M. Moss#, Marsha Gerdes§, David R. Jobes**, and Gil Wernovsky*, parallel

From the Divisions of * Cardiology, Dagger  Neurology, and § Neonatology at Children's Hospital of Philadelphia; Departments of parallel  Pediatrics and  Neurology at the University of Pennsylvania School of Medicine; # Department of Psychology at Children's Seashore House; and ** Department of Anesthesiology at the Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania.

Objectives.  The purposes of this study are to describe the quality of life and cognitive function in school-aged children who have undergone staged palliation for hypoplastic left heart syndrome (HLHS), and to identify factors that are predictive of neurodevelopmental outcome in this population.

Methods.  School-aged survivors with HLHS who had undergone palliative surgery 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 compared with the larger (remote) group of questionnaire respondents to determine whether results may be generalizable to the entire HLHS population. Potential predictors of neurologic and cognitive outcome were tested for their association with test scores using multivariate regression analysis.

Results.  Questionnaire results were obtained from 115 of 138 eligible children (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). The 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 some form of special education. Chronic medication usage was common (64%); the incidence of medical complications was comparable to that previously reported in children with Fontan physiology. Cognitive testing of the local group demonstrated a median full scale IQ of 86 (range: 50-116). Mental retardation (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 cohort of survivors was lower than that in the general population.  Key words:  hypoplastic left heart syndrome, neurodevelopmental outcome, quality of life.




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