Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Multicenter Study
. 2025 Sep 2;86(9):640-655.
doi: 10.1016/j.jacc.2025.06.051.

Prevalence and Predictors of Neurocognitive Dysfunction in Adults With Congenital Heart Disease: Results From MINDS-ACHD

Scott Cohen  1 , Michelle Gurvitz  2 , Kristin M Burns  3 , Olivia Wheaton  4 , Laura G Umfleet  5 , Nicole Brown  6 , Candice K Silversides  7 , Lauryn Dugan  8 , Mary E Stumpf  9 , Timothy B Cotts  10 , Peter R Ermis  11 , Susan M Jameson  12 , Stephanie Gaydos  13 , Arvind Hoskoppal  14 , Ian Lindsay  15 , Larry W Markham  9 , Stephanie Fuller  16 , Fred H Rodriguez 3rd  17 , Gayatri Ranganathan  4 , Felicia Trachtenberg  4 , Ali N Zaidi  18 ; PHN and AARCC Investigators
Affiliations
Multicenter Study

Prevalence and Predictors of Neurocognitive Dysfunction in Adults With Congenital Heart Disease: Results From MINDS-ACHD

Scott Cohen et al. J Am Coll Cardiol. .

Abstract

Background: Adults with congenital heart disease (ACHD) undergo repeated cardiac interventions, develop cardiac complications including atrial fibrillation, heart failure, and stroke, and may have genetic vulnerabilities for neurocognitive deficits (NCDs). However, NCDs have not been well studied in this population.

Objectives: The aim of this study was to examine the prevalence and risk factors associated with NCDs in young adults with moderate and severe complex congenital heart disease (CHD).

Methods: This was a prospective study of objective (cognitive battery of the National Institutes of Health [NIH] Toolbox) and subjective (NeuroQOL Item Bank v2) measures of neurocognitive function in ACHD patients (ages 18-30 years) from 14 North American ACHD centers. Multivariable regression was used to examine predictors of neurocognitive function.

Results: Overall, 467 ACHD participants were enrolled (82 dextro-transposition of the great arteries, 123 tetralogy of Fallot, 132 single ventricle, and 130 other CHD). The mean age was 24.5 years, 44% were male, 81% were White, and 78% had NYHA functional class I. For the total cohort, 34% scored at least 1 standard deviation below the normative mean on the NIH Toolbox Total Composite Score and 22% scored at least 1 standard deviation below the normative mean on the NeuroQOL. Education and NYHA functional class were independently associated with NIH Toolbox scores. Education, body mass index, atrial arrhythmias, depression, and anxiety were independently associated with NeuroQOL scores.

Conclusions: NCDs are prevalent in young adults with moderate and severely complex CHD, with approximately one-third having objective NCDs and one-fifth subjective NCDs. These findings emphasize the importance of integrating neurocognitive assessments into routine ACHD care.

Keywords: congenital heart disease; neurocognitive dysfunction.

PubMed Disclaimer

Conflict of interest statement

Funding Support and Author Disclosures Funding for this study was provided by the National Heart, Lung, and Blood Institute through the Pediatric Heart Network, supporting the clinical sites and the data coordinating center, including grants HL135646, HL135665, HL135666, HL135678, HL135680, HL135682, HL135683, HL135685, HL135689, and HL135691. The views expressed in this article are those of the authors and do not necessarily represent the views of the National Heart, Lung, and Blood Institute; the National Institutes of Health; or the U.S. Department of Health and Human Services. The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

References

    1. Gilboa SM, et al. , Congenital Heart Defects in the United States: Estimating the Magnitude of the Affected Population in 2010. Circulation, 2016. 134(2): p. 101–9. - PMC - PubMed
    1. Hoffman JI and Kaplan S, The incidence of congenital heart disease. J Am Coll Cardiol, 2002. 39(12): p. 1890–900. - PubMed
    1. Bellinger DC, et al. , Developmental and neurologic status of children after heart surgery with hypothermic circulatory arrest or low-flow cardiopulmonary bypass. N Engl J Med, 1995. 332(9): p. 549–55. - PubMed
    1. Bellinger DC, et al. , Adolescents with d-transposition of the great arteries corrected with the arterial switch procedure: neuropsychological assessment and structural brain imaging. Circulation, 2011. 124(12): p. 1361–9. - PMC - PubMed
    1. Marino BS, et al. , Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation, 2012. 126(9): p. 1143–72. - PubMed

Publication types

LinkOut - more resources