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

Objective: To compare neurodevelopmental outcome (NDO) in patients with hyp oplastic left heart syndrome (HLHS), other functional single ventricle lesi ons, and the standard population and to investigate predictors of NDO in th e population of children with functional single ventricle (FSV). Study design: A time- and age-defined cohort of patients with the Fontan ci rculation nas recruited to participate in neurodevelopmental testing, behav ioral evaluation, and imaging of the central nervous system. The Wechsler I ntelligence test was the primary measure of NDO. Analysis included comparis on of patients with HLHS with other patients with functional single ventric les. 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 grou p had significantly lower scores than the non-HLHS subgroup, neither subgro up scored significantly different from the standard population on the Wechs ler Scares. Socioeconomic status, circulatory arrest, and perioperative sei zures 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 general ly in the normal range.