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

Rachel E Siciliano1, Lexa K Murphy1, Kemar V Prussien1

  • 1Department of Psychology and Human Development, Vanderbilt University, Nashville, TN, USA.

Insights

Children with hypoplastic left heart syndrome (HLHS) experience significant cognitive deficits, particularly in processing speed and attention, impacting their development. Further research is crucial to understand these deficits and develop targeted interventions for affected youth.

Area of Science:

  • Pediatric Cardiology
  • Neurodevelopmental Pediatrics
  • Cognitive Psychology

Background:

  • Improved survival rates for hypoplastic left heart syndrome (HLHS) have shifted focus to long-term neurodevelopmental outcomes.
  • Previous research primarily assessed broad cognitive abilities in young children, leaving a gap in understanding specific cognitive functions in older youth with HLHS.

Purpose of the Study:

  • To investigate specific cognitive functions and attention in youth aged 8 to 16 years with HLHS.
  • To compare cognitive performance in HLHS youth to normative data.
  • To explore potential correlations between cognitive deficits, attention problems, and age.

Main Methods:

  • Pilot study involving 20 youth (ages 8-16) diagnosed with HLHS.
  • Cognitive function assessed using the Wechsler Intelligence Scale for Children - Fifth Edition (WISC-V) and NIH Toolbox Cognition Battery (NTCB).
  • Parental reports on behavior collected via the Child Behavior Checklist.

Main Results:

  • HLHS youth demonstrated significantly lower scores than normative means across multiple WISC-V indices (Full Scale IQ, Verbal Comprehension, Visual Spatial, Working Memory, Processing Speed) and the NTCB Fluid Cognition Composite.
  • Effect sizes for these cognitive deficits ranged from medium to large.
  • Attention problems were significantly associated with poorer cognitive outcomes, and older age correlated with lower visual-spatial scores.

Conclusions:

  • Youth with HLHS exhibit widespread and significant cognitive deficits, extending beyond broad measures.
  • Attention difficulties represent a substantial challenge for this population.
  • Comprehensive cognitive assessments are vital for identifying specific deficits to guide targeted interventions, with consideration for age and disease-related factors in future research.