Cognitive and Attentional Function in Pediatric Hypoplastic Left Heart Syndrome

Abstract

Despite surgical palliation, children with hypoplastic left heart syndrome (HLHS) have compromised cardiac functioning and increased risk for cognitive deficits. Yet, little is known about detailed profiles of cognitive function in these children. First, I quantitatively review data from 13 studies of cognitive function in children with HLHS. The meta-analysis revealed large effects for Full Scale IQ and Performance IQ, and a medium effect for Verbal IQ. Meta-regression analyses of effect size on child age revealed a significant effect for Full Scale IQ, indicating a loss of 1.1 IQ points with each year of sample age. FSIQ deficits may reflect chronic brain injury or failure to make expected gains with age. Second, I examine specific cognitive indices and attention problems in a sample of children with HLHS, and explore relations with child age and risk factors. Children with HLHS (n = 20), aged 8 to 16 years, scored lower than the normative mean on Full Scale IQ, Verbal Comprehension, Visual Spatial, and Processing Speed indices, with medium and large effects. Effects for Fluid Reasoning and Working Memory indices were nonsignificant. There were large effects for significantly elevated self- and parent-reported attention problems. Child age was related to poorer visual spatial scores. These findings highlight the importance of early intervention in this population, the need for longitudinal studies of more specific domains of cognitive function and potential moderators, and the importance of assessing specific domains of cognitive function and investigating age and disease factors as potential correlates in larger sample.