Cited by 113Congenital Heart Disease StudiesCongenital heart defects researchCardiovascular Conditions and Treatments

Adolescents with tetralogy of Fallot: neuropsychological assessment and structural brain imaging

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Bellinger, David C., Rivkin, Michael J., DeMaso, David, Robertson, Richard L., Stopp, Christian, Dunbar-Masterson, Carolyn, Wypij, David, Newburger, Jane W.·2015·Cardiology in the Young

Abstract

Background: Few data are available on the neuropsychological, behavioural, or structural brain imaging outcomes in adolescents who underwent corrective surgery in infancy for tetralogy of Fallot. Methods: In this single-centre cross-sectional study, we enrolled 91 adolescents (13–16 years old) with tetralogy of Fallot and 87 referent subjects. Assessments included tests of academic achievement, memory, executive functions, visual-spatial skills, attention, and social cognition, as well as brain magnetic resonance imaging. Results: Genetic abnormalities or syndromes were present in 25% of tetralogy of Fallot patients, who had markedly greater neuropsychological morbidities than did patients without a syndrome. However, even patients without a syndrome performed significantly worse than the referent group or population norms in all of the neuropsychological domains assessed. In multivariable regression in those without a genetic/phenotypic syndrome, the strongest predictors of adverse late neurodevelopmental outcomes included a greater number of complications at the first operation, more total surgical complications across all operations, and occurrence of post-operative seizures. The presence of at least one abnormality on structural magnetic resonance imaging was more frequent in tetralogy of Fallot patients than the referent group (42% versus 8%). Conclusions: Adolescents with tetralogy of Fallot are at increased neurodevelopmental risk and would benefit from ongoing surveillance and educational supports even after childhood.

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  • A genetic/phenotypic syndrome constitutes a principal determinant of neurodevelopmental impairments, and non-syndromic TOF patients also scored lower than healthy peers in 3 of 4 studies.

    “a genetic/phenotypic syndrome constitutes a principal determinant of neurodevelopmental impairments”

    From Neurodevelopmental Outcomes in Tetralogy of Fallot: A Systematic Review (2022) · cites this paper as [24]

    The Discussion reports that adolescents with a genetic/phenotypic diagnosis performed significantly worse in most respects than those without one, supporting a strong association with neuropsychological impairment. It does not establish that the syndrome is a principal determinant or a cause of those impairments. (LLM, conf=0.82)

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  • In TOF adolescents aged 13-16 years, patients scored lower than healthy peers in executive functions, visual-spatial skills, memory, attention, academic achievement, and social cognition, regardless of genetic status.

    “In all of the aforementioned domains, patients scored lower than their healthy peers, regardless of their genetic status.”

    From Neurodevelopmental Outcomes in Tetralogy of Fallot: A Systematic Review (2022) · cites this paper as [24]

    The Executive Functions, Visual-spatial Skills, Memory, Attention, and Social Cognition sections report poorer results in adolescents with tetralogy of Fallot both with and without a genetic/phenotypic syndrome. The Academic Achievement section reports slightly lower reading and math scores in those without a syndrome, but those differences from population norms were not significant. The Subjects section also says healthy referents were used only for tests lacking national norms; most cited comparisons were against population norms, not directly against healthy peers. Page numbers were unavailable in our copy. (LLM, conf=0.85)

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Research works printed but not linked: 3

  1. [2]2. Loffredo CA. Epidemiology of cardiovascular malformations: prevalence and risk factors. Am J Med Genet 2000; 97: 319-325.
  2. [15]15. The Psychological Corporation. Wechsler Individual Achievement Test-Second Edition. The Psychological Corporation, San Antonio, 2002.
  3. [28]28. Bellinger DC, Wypij D, Rivkin MJ, et al. Adolescents with d-transposition of the great arteries corrected with the arterial switch procedure. Circulation 2011; 124: 1361-1369.

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Authors
Bellinger, D. C., Rivkin, M. J., DeMaso, D., Robertson, R. L., Stopp, C., Dunbar-Masterson, C., Wypij, D., & Newburger, J. W.
Affiliations
Boston Children's Hospital (US)
Venue
Cardiology in the Young
Published
2014-02-11
Volume
25
Issue
2
Pages
338-347
ISSN
  • 1047-9511 (ISSN-L)
  • 1467-1107
OpenAlex
W2333963874
Cited by
113
References
30
Citations by year
2026: 6 · 2025: 3 · 2024: 11 · 2023: 10 · 2022: 11 · 2021: 9 · 2020: 12 · 2019: 13 · 2018: 11 · 2017: 12 · 2016: 9 · 2015: 4 · 2014: 2
FWCI
6.82
Type
journal article
Language
en
Open access
Locations
  • Cardiology in the Young (publishedVersion)
  • PubMed (publishedVersion)
  • PubMed Central (acceptedVersion)
Funders
National Heart, Lung, and Blood Institute (P50HL074734, HL-74734, P50 HL74734)
Topics
  • Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Congenital heart defects research
  • Health Sciences › Medicine › Radiology, Nuclear Medicine and Imaging › Cardiovascular Conditions and Treatments
Keywords
tetralogy of Fallot, adolescents, neuropsychological outcomes, neurodevelopmental outcomes, genetic syndromes, surgical complications, postoperative seizures, structural brain abnormalities, executive function, memory, academic performance, social cognition, attention, visuospatial ability
MeSH terms
Achievement, Adolescent, Attention, Brain, Cognition Disorders, Female, Humans, Magnetic Resonance Imaging, Male, Memory, Neuropsychological Tests, Regression Analysis, Social Perception, Space Perception, Tetralogy of Fallot, Survivors, Executive Function, Social Skills

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