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Executive Function in Children and Adolescents with Critical Cyanotic Congenital Heart Disease

Adam R Cassidy1, Matthew T White1, David R DeMaso1

  • 11Department of Psychiatry,Boston Children's Hospital,Harvard Medical School,Boston,Massachusetts.

Journal of the International Neuropsychological Society : JINS
|December 10, 2014
PubMed

Insights

Children with critical congenital heart disease (CHD) face significant executive function (EF) deficits. These deficits, impacting problem-solving and self-regulation, are more prevalent and severe with greater CHD complexity.

Area of Science:

  • Neuroscience
  • Pediatrics
  • Cardiology

Background:

  • Critical congenital heart disease (CHD) poses risks to neurodevelopment in children and adolescents.
  • Executive function (EF) deficits are a known concern in pediatric populations with complex medical conditions.

Purpose of the Study:

  • To compare executive function (EF) outcomes in children and adolescents with three types of severe CHD versus healthy controls.
  • To identify specific EF profiles associated with dextro-transposition of the great arteries (TGA), tetralogy of Fallot (TOF), and single-ventricle anatomy requiring Fontan procedure (SVF).

Main Methods:

  • A cohort of 463 children/adolescents with severe CHD (TGA, TOF, SVF) and 111 controls (ages 10-19) were evaluated.
  • Laboratory-based (D-KEFS) and informant-based (BRIEF) assessments were used to measure EF skills.
  • EF impairment rates and specific cognitive profiles were compared across groups.

Main Results:

  • Children with CHD exhibited nearly double the rate of EF impairment (75-81%) compared to controls (43%).
  • All CHD groups showed deficits in flexibility/problem-solving and verbal EF; TOF and SVF groups also had visuospatial EF impairments.
  • Informant reports revealed distinct metacognitive and self-regulatory concerns unique to each CHD group.

Conclusions:

  • Severe CHD significantly threatens executive function development in children and adolescents.
  • Greater CHD severity correlates with poorer EF outcomes.
  • Understanding these cognitive vulnerabilities can guide early identification and tailored interventions for optimal neurodevelopmental support.

Keywords:
BRIEF: Behavior Rating Inventory of Executive Function.CHD: congenital heart diseaseCongenital heart defectsD-KEFS: Delis-Kaplan Executive Function SystemExecutive functionFontan procedureHLHS: hypoplastic left heart syndromeSVF: single-ventricle cardiac conditions requiring Fontan procedureSingle-ventricleTGA: dextro-transposition of the great arteriesTOF: tetralogy of FallotTetralogy of FallotTransposition of great vessels

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