Cognitive disability is the most prevalent disability type among young adults with congenital heart defects (CHD), reported by more than a quarter of young adults with CHD in CH STRONG (Congenital Heart Survey To Recognize Outcomes, Needs, and well-beinG).¹ However, the long-term outcomes of adults with CHD and cognitive disabilities are not well understood. Therefore, we compared the socioeconomic and healthcare outcomes among adults (aged 19 to 38 years) with CHD with and without cognitive disabilities in CH STRONG and described the socioeconomic characteristics associated with the receipt of disability benefits among adults with CHD and cognitive disabilities.

In 2016 to 2019, surveys were collected from adults with CHD born between 1980 and 1997 and identified from 3 state birth defect registries (Arizona, Arkansas, and Metro Atlanta, Georgia).² Questions covered sociodemographics, health care, receipt of disability benefits (not including Medicaid), and the Department of Health and Human Services standard assessment for functional cognitive disability (“Because of a physical, mental, or emotional condition, do you have serious difficulty concentrating, remembering, or making decisions?”). For this analysis, adults with chromosomal anomalies (including Down syndrome) were excluded. To address nonresponse (CH STRONG survey response rate = 23.9%), we standardized the cognitive disability prevalence to the site, birth year, sex assigned at birth, maternal race/ethnicity, and CHD anatomic severity distribution of the CH STRONG−eligible population (n = 9,312). The adjusted prevalence ratios (aPRs) from Poisson regression described the associations between the outcomes and cognitive disability. Among those with cognitive disabilities, aPRs identified the socioeconomic characteristics associated with ever receiving disability benefits, and chi-square tests compared the healthcare outcomes by receipt of disability benefits. All analyses were conducted using SAS-callable SUDAAN version 11.0 (RTI International; Research Triangle Park, North Carolina).

Of the 1,375 participants included in the analysis, 348 (25.8% after standardization) reported having a cognitive disability (Figure 1). Adults with CHD and cognitive disabilities, compared with adults with CHD without cognitive disabilities, more commonly had a high school education or less, ever received special education services, and worked part-time or less in the previous year (Table 1). They were also more likely publicly insured or uninsured, even when adjusting for education and employment (aPRs = 1.4 and 1.7, respectively). A larger percentage of adults with CHD and cognitive disabilities identified cost as a reason for not seeing a doctor in the previous year, even among those with private or public insurance (aPR = 1.6). In addition, 19.1% of adults with CHD and cognitive disabilities had no usual place for health care. Adults with CHD and cognitive disabilities more often had ≥8 outpatient and ≥2 emergency department visits in the previous year than adults with CHD without cognitive disabilities. Only 62.9% of adults with CHD and cognitive disabilities and 52.5% without cognitive disabilities had cardiology care in the past 5 years.

Among adults with CHD and cognitive disabilities, 37.8% reported ever receiving disability benefits. Those who had a high school education or less, ever received special education, were unemployed, and had public insurance were more likely to receive disability benefits than their respective counterparts (aPRs = 2.2 to 4.7). Those who received disability benefits less often postponed care because of cost and more often had cardiology care in the past 5 years (both chi-square p <0.01).

In summary, adults with CHD and cognitive disabilities had a lower level of education and rate of employment and were more commonly publicly insured or uninsured than adults with CHD without cognitive disabilities. They more often delayed care because of cost, even if insured. Almost 40% of adults with CHD and cognitive disabilities had not seen a cardiologist in the past 5 years, and only 1/3 reported ever receiving disability benefits. Adults with CHD and cognitive disabilities reported more healthcare visits and emergency care than those without cognitive disabilities. Our study limitations include the following: causality cannot be determined, cognitive disability was self-reported and not clinically confirmed, and severity and chronicity of cognitive disability were not assessed.

Several possible options exist to care for and potentially improve the health and socioeconomic outcomes of adults with CHD and cognitive disabilities.³ Neurocognitive screening and evaluation can determine the state of the disability and appropriate referral services. The guidelines for the clinical management of adults with CHD encourage neurodevelopmental testing to direct therapies that enhance academic, behavioral, psychosocial, and adaptive functioning.⁴ Other strategies to increase healthcare accessibility and affordability for people with disabilities include telemedicine (with careful consideration for patient limitations), coordinated care, and shared decision-making.^5,6 Finally, improving regular use of both primary and cardiology care could result in less emergency care.^7–9