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

Abstract

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. 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). 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. 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. 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.