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Brain in Congenital Heart Disease Across the Lifespan: The Cumulative Burden of Injury
Open accessAbstract
The number of patients surviving with congenital heart disease (CHD) has soared over the last 3 decades. Adults constitute the fastest-growing segment of the CHD population, now outnumbering children. Research to date on the heart-brain intersection in this population has been focused largely on neurodevelopmental outcomes in childhood and adolescence. Mutations in genes that are highly expressed in heart and brain may cause cerebral dysgenesis. Together with altered cerebral perfusion in utero, these factors are associated with abnormalities of brain structure and brain immaturity in a significant portion of neonates with critical CHD even before they undergo cardiac surgery. In infancy and childhood, the brain may be affected by risk factors related to heart disease itself or to its interventional treatments. As children with CHD become adults, they increasingly develop heart failure, atrial fibrillation, hypertension, diabetes mellitus, and coronary disease. These acquired cardiovascular comorbidities can be expected to have effects similar to those in the general population on cerebral blood flow, brain volumes, and dementia. In both children and adults, cardiovascular disease may have adverse effects on achievement, executive function, memory, language, social interactions, and quality of life. Against the backdrop of shifting demographics, risk factors for brain injury in the CHD population are cumulative and synergistic. As neurodevelopmental sequelae in children with CHD evolve to cognitive decline or dementia during adulthood, a growing population of CHD can be expected to require support services. We highlight evidence gaps and future research directions.
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Children with critical CHD are reported to have neurodevelopmental impairments.
“many experience behavioral, emotional, cognitive and motor impairment suggesting widespread brain dysfunction that continues into adulthood (3–5).”
From Neonatal Brain Injury and Timing of Neurodevelopmental Assessment in Patients With Congenital Heart Disease (2018) · cites this paper as [4]
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Metadata
- Authors
- Marelli, A., Miller, S. P., Marino, B. S., Jefferson, A. L., & Newburger, J. W.
- Affiliations
- Northwestern University (US)
- Venue
- Circulation
- Published
- 2016-05-17
- Volume
- 133
- Issue
- 20
- Pages
- 1951-1962
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- PMC
- PMC5519142
- OpenAlex
- W2408520533
- Cited by
- 359
- References
- 109
- Citations by year
- 2026: 26 · 2025: 37 · 2024: 42 · 2023: 40 · 2022: 44 · 2021: 33 · 2020: 48 · 2019: 31 · 2018: 27 · 2017: 26 · 2016: 3
- FWCI
- 27.17
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Circulation (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- TSpace (University of Toronto) (submittedVersion)
- Funders
- National Institute on Aging (K24AG046373)
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (U54HD083211, U54HD090255)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Congenital Heart Disease Studies
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cardiovascular and Diving-Related Complications
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Congenital heart defects research
- Keywords
- congenital heart disease, brain, neurodevelopmental outcomes, cerebral dysgenesis, cerebral perfusion, brain immaturity, neonates, heart failure, atrial fibrillation, hypertension, dementia, cognitive decline, executive function, quality of life, cardiovascular comorbidity, lifespan
- MeSH terms
- Brain, Brain Injuries, Child, Child Development, Heart Defects, Congenital, Humans, Longevity, Risk Factors, Cost of Illness
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