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

Cassidy, Adam R.; White, Matthew T.; DeMaso, David R.; Newburger, Jane W.; Bellinger, David C. · 2014 · OpenAlex-citations

DOI: 10.1017/s1355617714001027

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Summary

This study investigates executive function (EF) deficits in children and adolescents with critical cyanotic congenital heart disease (CHD), aiming to determine if specific cardiac diagnoses are associated with distinct patterns of cognitive vulnerability. The research addresses the known risk of neurodevelopmental impairment in severe CHD, driven by factors such as prenatal atypical brain development, hypoxic-ischemic injury, and surgical interventions. The primary objective was to compare EF outcomes across three CHD subgroups—dextro-transposition of the great arteries (TGA), tetralogy of Fallot (TOF), and single-ventricle anatomy requiring the Fontan procedure (SVF)—against a control group. The study utilized data from three large-scale, single-center longitudinal studies at Boston Children’s Hospital, involving 463 participants with CHD (139 TGA, 68 TOF, 145 SVF) and 111 typically developing controls, aged 10–19 years. Executive function was assessed using two primary instruments: the Delis-Kaplan Executive Function System (D-KEFS), a laboratory-based battery measuring verbal fluency, design fluency, sorting, word context, and tower planning; and the Behavior Rating Inventory of Executive Function (BRIEF), which gathered parent, teacher, and self-report ratings of real-world EF skills. Statistical analyses included profile analysis to compare score patterns across groups and logistic regression to determine impairment rates, controlling for covariates such as socioeconomic status, age, sex, and race. Results indicated that EF impairment rates on D-KEFS measures were nearly twice as high in CHD groups (75–81%) compared to controls (43%). Distinct EF profiles emerged among the CHD subgroups. Deficits in flexibility, problem-solving, and verbally mediated EF skills were present in all three CHD groups. However, visuo-spatially mediated EF abilities were impaired in the TOF and SVF groups but remained preserved in the TGA group, which performed similarly to controls on these specific tasks. Additionally, BRIEF ratings revealed unique patterns of metacognitive and self-regulatory concerns across informants. While parents and teachers reported significant EF problems, self-report ratings did not reflect the same degree of severity, suggesting that individuals with CHD may underestimate their functional challenges. The findings demonstrate that critical cyanotic CHD poses a serious threat to executive function development, with greater disease severity associated with worse outcomes. The identification of distinct cognitive profiles based on cardiac diagnosis suggests that neurodevelopmental risks are not uniform across CHD types. These results imply that early identification of specific cognitive and self-regulatory vulnerabilities is necessary to provide individualized supports, potentially promoting optimal neurodevelopment in this high-risk population.

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• executive function