Neurodevelopmental outcome of patients after the Fontan operation: A comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions

• Published in: The Journal of pediatrics 2000-11, Vol.137 (5), p.646-652
• Main Authors: Goldberg, Caren S., Schwartz, Edward M., Brunberg, James A., Mosca, Ralph S., Bove, Edward L., Schork, M.Anthony, Stetz, Sharon P., Cheatham, John P., Kulik, Thomas J.
• Format: Article
• Language: English
• Subjects: Biological and medical sciences; Cardiology. Vascular system; Central Nervous System - pathology; Child Behavior; Child Development; Child, Preschool; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Developmental Disabilities; Female; Fontan Procedure; Heart; Humans; Hypoplastic Left Heart Syndrome - surgery; Infant; Intelligence; Magnetic Resonance Imaging; Male; Medical sciences; Neurologic Examination; Psychomotor Performance; Treatment Outcome; Ventricular Dysfunction - surgery; Wechsler Scales
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1067/mpd.2000.108952

Objective: To compare neurodevelopmental outcome (NDO) in patients with hypoplastic left heart syndrome (HLHS), other functional single ventricle lesions, and the standard population and to investigate predictors of NDO in the population of children with functional single ventricle (FSV). Study design: A time- and age-defined cohort of patients with the Fontan circulation was recruited to participate in neurodevelopmental testing, behavioral evaluation, and imaging of the central nervous system. The Wechsler Intelligence test was the primary measure of NDO. Analysis included comparison of patients with HLHS with other patients with functional single ventricles. Other potential clinical predictors of NDO were investigated. Results: The mean Full Scale Wechsler Intelligence score was 101.4 ± 5.4. For the HLHS subgroup the mean Full Scale Wechsler score was 93.8 ± 7.3, and for the non-HLHS subgroup it was 107.0 ± 7.0. Although the HLHS group had significantly lower scores than the non-HLHS subgroup, neither subgroup scored significantly different from the standard population on the Wechsler Scales. Socioeconomic status, circulatory arrest, and perioperative seizures also were predictive of neurodevelopmental outcome. Conclusion: Neurodevelopmental and behavioral outcome in patients who have undergone the Fontan procedure including patients with HLHS is good in the preschool and early school years, with Wechsler Intelligence scores generally in the normal range. (J Pediatr 2000;137:646-52)