Is cardiac diagnosis a predictor of neurodevelopmental outcome after cardiac surgery in infancy?
Open accessAbstract
Objectives To determine whether a cardiac diagnosis is a predictor of neurodevelopmental outcomes after infant cardiac surgery.Methods Infants with ventricular septal defect (VSD), tetralogy of Fallot (TOF), transposition of the great arteries (TGA), and hypoplastic left heart syndrome (HLHS) in a study of apolipoprotein E (APOE) polymorphisms, and neurodevelopmental outcome underwent neurodevelopmental and genetic evaluation at 4 years of age. The domains tested included cognition, language, speech, memory, executive function, visual-motor, fine motor, and reading and math skills.Results Testing was completed in 178 patients with normal genetic evaluations: VSD (n = 26), TOF (n = 44), TGA (n = 41), and HLHS (n = 67). No differences were found in gestational age, ethnicity, APOE genotype, socioeconomic status, or maternal education among groups. Patient age at the first surgery was significantly lower for patients with TGA and HLHS compared with those with TOF and VSD. The postoperative length of stay was significantly longer for HLHS than all other groups and for TGA compared with TOF and VSD. HLHS correlated significantly with the use of deep hypothermic circulatory arrest and multiple operations. The mean scores for each domain were within normal limits for all groups. Compared with the other patients, those with HLHS had significantly lower scores for cognition, fine motor skills, executive function, and math skills. No significant differences were found among the TGA, TOF or VSD patients for any domain. Significant impairments in at least 1 domain were identified in 8% (2/25) of patients with VSD, 20% (8/41) with TOF, 17% (7/41) with TGA, and 18% (12/65) with HLHS. After correction for the demographic, preoperative, and operative variables, no significant differences were found among the groups for any domain.Conclusions The mean scores for the neurodevelopmental outcomes domains tested were in the normal range for preschool children with no recognized genetic syndromes after surgery for VSD, TOF, TGA, and HLHS. In each diagnostic group, the number of children with impairments in at least 1 domain increased compared with the general population. Unadjusted neurodevelopmental outcomes for HLHS were lower for cognition, fine motor skills, executive function, and math skills compared with the other patients. After correction for the demographic, preoperative, and operative variables, no significant differences were found among the groups for any domain. The specific cardiac diagnosis determines a large portion of the variation in these covariates. Therefore, although HLHS did predict for poorer outcomes in some domains, it did not add predictive power to the other factors considered.
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The interplay between disease complexity and neurodevelopment is present in TOF, similarly to other severe CHDs.
“Thus, the interplay between disease complexity and neurodevelopment is also present in TOF, similarly to patients with other severe CHDs, including single ventricle heart disease and transposition of the great arteries [5, 7, 21].”
From Early Evaluation and the Effect of Socioeconomic Factors on Neurodevelopment in Infants with Tetralogy of Fallot. (2021) · cites this paper as [21]
The Results (Neurodevelopmental Testing; page unavailable) report impairments in TOF as well as the other diagnostic groups, and lower unadjusted scores in HLHS. But the study does not establish that disease complexity predicts neurodevelopmental outcome within TOF; after adjustment, it found no significant differences among the diagnostic groups. (LLM, conf=0.82)
Experimental — accuracy not yet measuredIn 4-year-old TOF children, mean unadjusted results for all ND domains were within the population's anticipated range, but moderate to severe impairment in at least one domain was higher than expected.
“mean unadjusted results derived from the assessment of all ND domains were within the overall population’s anticipated range”
From Neurodevelopmental Outcomes in Tetralogy of Fallot: A Systematic Review (2022) · cites this paper as [18]
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- Authors
- Gaynor, J. W., Gerdes, M., Nord, A. S., Bernbaum, J., Zackai, E., Wernovsky, G., Clancy, R. R., Heagerty, P. J., Solot, C. B., McDonald-McGinn, D., & Jarvik, G. P.
- Affiliations
- Children's Hospital of Philadelphia (US)
- University of Washington (US)
- Corresponding author
- Gaynor, J. William
- ORCID
- Venue
- The Journal of Thoracic and Cardiovascular Surgery
- Publisher
- Elsevier BV
- Published
- 2010-12
- Volume
- 140
- Issue
- 6
- Pages
- 1230-1237
- ISSN
- 0022-5223 (ISSN-L)
- PMC
- PMC3278908
- OpenAlex
- W2034386674
- Cited by
- 88
- References
- 16
- Citations by year
- 2026: 1 · 2025: 2 · 2023: 7 · 2022: 3 · 2021: 6 · 2020: 10 · 2019: 7 · 2018: 3 · 2017: 8 · 2016: 7 · 2015: 3 · 2014: 13 · 2013: 6 · 2012: 8
- FWCI
- 4.19
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Journal of Thoracic and Cardiovascular Surgery (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Heart, Lung, and Blood Institute (R01HL071834)
- Topics
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Health Sciences › Medicine › Radiology, Nuclear Medicine and Imaging › Cardiovascular Conditions and Treatments
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Congenital heart defects research
- Keywords
- neurodevelopmental outcomes, infant cardiac surgery, congenital heart disease, ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, hypoplastic left heart syndrome, cognitive impairment, executive function, fine motor skills, APOE polymorphism, deep hypothermic circulatory arrest, multiple operations, postoperative hospital stay, preschool children
- MeSH terms
- Child Development, Child, Preschool, Cognition Disorders, Female, Heart Defects, Congenital, Humans, Hypothermia, Induced, Infant, Length of Stay, Male, Motor Skills, Nervous System Diseases, Neuropsychological Tests, Predictive Value of Tests, Prospective Studies, Risk Factors, Treatment Outcome, Apolipoprotein E2, Executive Function
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