Cognitive and Attentional Function in Children with Hypoplastic Left Heart Syndrome: A Pilot Study
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Cognitive and Attentional Function in Children with Hypoplastic Left Heart Syndrome: A Pilot Study Rachel E. Siciliano1 · Lexa K. Murphy1 · Kemar V. Prussien1 · Lauren M. Henry1 · Kelly H. Watson1 · Niral J. Patel2 · Chelsea A. Lee2 · Colleen M. McNally1 · Larry W. Markham3 · Bruce E. Compas1 · Lori C. Jordan2 Accepted: 10 November 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract While survival for children with hypoplastic left heart syndrome (HLHS) has improved, compromised cardiac output and oxygen delivery persist, and children show cognitive deficits. Most research has assessed young children on broad cognitive indices; less is known about specific indices in older youth. In this pilot study, cognitive function and attention in youth ages 8 to 16 years with HLHS (n = 20) was assessed with the Wechsler Intelligence Scale for Children – Fifth Edition (WISC-V) and NIH Toolbox Cognition Battery (NTCB); parents completed the Child Behavior Checklist. Children scored significantly lower than normative means on the WISC-V Full Scale IQ, Verbal Comprehension, Visual Spatial, Working Memory, and Processing Speed indices, and the NTCB Fluid Cognition Composite; effect sizes ranged from medium to large. Attention problems had a large significant effect. Child age corresponded to lower visual spatial scores. Findings highlight the importance of assessing multiple cognitive indices for targeted intervention and investigating age and disease factors as potential correlates in larger samples. Keywords Congenital heart disease · HLHS · Cognition · Attention · Risk factors The prevalence of congenital heart disease (CHD), particularly severe forms, has increased in children and adults in recent years due to improved diagnosis and advances in surgical techniques and postoperative management (Marelli et al., 2014). Hypoplastic left heart syndrome (HLHS) is a specific type of “single ventricle” congenital defect characterized by the inability to pump oxygen-rich blood to the body and is arguably the most severe form of CHD (Benjamin et al., 2018; Javed et al., 2019). Standard surgical techniques for HLHS palliation include three procedures (Norwood, Bi-Directional Glenn, and Fontan operations) to bypass the underdeveloped left side of the heart. * Lori C. Jordan [email protected] 1
Department of Psychology and Human Development, Vanderbilt University, Nashville, TN, USA
2
Department of Pediatrics, Division of Pediatric Neurology, Vanderbilt University Medical Center, 2200 Children’s Way, Nashville, TN DOT 11212, USA
3
Department of Pediatrics, Division of Pediatric Cardiology, Indiana University School of Medicine, Indianapolis, IN, USA
Postoperatively, children continue to have compromised cardiac output, reduced systemic oxygen delivery, high systemic oxygen extraction, and anaerobic end-organ dysfunction (Benjamin et al., 2018; Feinstein et al., 2012). As 90% of children with HLHS now survive past infancy, the importance of tracking their outcomes into school-age years an
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