Cognitive and Attentional Function in Children with Hypoplastic Left Heart Syndrome: A Pilot Study


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.

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This work was supported by the Vanderbilt Department of Pediatrics via the Turner-Hazinski grant program, a training grant from the National Institute of Mental Health (T32-MH18921), a gift from Rhodes and Patricia Hart, and an anonymous donor.

Author information




Study Conceptualization: LCJ, LKM, BEC; Methodology: LCJ, LKM, BEC; Formal analysis and investigation: RES, LKM, KVP, LMH, NJP, CAL; CMM, BEC, LCJ; Writing: RES, LWM, BEC, LCJ; Funding acquisition: LCJ, BEC, KVP; Resources: LCJ; Supervision: LCJ, BEC.

Corresponding author

Correspondence to Lori C. Jordan.

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Conflict of interest

Rachel E. Siciliano, Lexa K. Murphy, Kemar V. Prussien, Lauren M. Henry, Kelly H. Watson, Niral J. Patel, Chelsea A. Lee, Colleen M. McNally, Larry W. Markham, Bruce E. Compas, and Lori C. Jordan declare that they have no conflict of interest.

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All procedures contributing to this work comply with the ethical standards of the relevant national guidelines on human experimentation and with the 1964 Helsinki Declaration and its later amendments and has been approved by the institutional review board of Vanderbilt University Medical Center.

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Siciliano, R.E., Murphy, L.K., Prussien, K.V. et al. Cognitive and Attentional Function in Children with Hypoplastic Left Heart Syndrome: A Pilot Study. J Clin Psychol Med Settings (2020).

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  • Congenital heart disease
  • HLHS
  • Cognition
  • Attention
  • Risk factors