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J Int Neuropsychol Soc. 2015 Jan;21(1):34-49. doi: 10.1017/S1355617714001027. Epub 2014 Dec 9.

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

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1Department of Psychiatry,Boston Children's Hospital,Harvard Medical School,Boston,Massachusetts.
2Department of Cardiology,Boston Children's Hospital,Harvard Medical School,Boston,Massachusetts.


Children and adolescents with critical cyanotic congenital heart disease (CHD) are at risk for deficits in aspects of executive function (EF). The primary aim of this investigation was to compare EF outcomes in three groups of children/adolescents with severe CHD and controls (ages 10-19 years). Participants included 463 children/adolescents with CHD [dextro-transposition of the great arteries (TGA), n=139; tetralogy of Fallot (TOF), n=68; and, single-ventricle anatomy requiring Fontan procedure (SVF), n=145] and 111 controls, who underwent laboratory and informant-based evaluation of EF skills. Rates of EF impairment on D-KEFS measures were nearly twice as high for CHD groups (75-81%) than controls (43%). Distinct EF profiles were documented between CHD groups on D-KEFS tasks. Deficits in flexibility/problem-solving and verbally mediated EF skills were documented in all three CHD groups; visuo-spatially mediated EF abilities were impaired in TOF and SVF groups, but preserved in TGA. Parent, teacher, and self-report ratings on the BRIEF highlighted unique patterns of metacognitive and self-regulatory concerns across informants. CHD poses a serious threat to EF development. Greater severity of CHD is associated with worse EF outcomes. With increased understanding of the cognitive and self-regulatory vulnerabilities experienced by children and adolescents with CHD, it may be possible to identify risks early and provide individualized supports to promote optimal neurodevelopment.


BRIEF: Behavior Rating Inventory of Executive Function.; CHD: congenital heart disease; Congenital heart defects; D-KEFS: Delis-Kaplan Executive Function System; Executive function; Fontan procedure; HLHS: hypoplastic left heart syndrome; SVF: single-ventricle cardiac conditions requiring Fontan procedure; Single-ventricle; TGA: dextro-transposition of the great arteries; TOF: tetralogy of Fallot; Tetralogy of Fallot; Transposition of great vessels

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