Adults With Congenital Heart Disease Have an Increased Prevalence of Autoimmunity

BACKGROUND: Adult congenital heart disease (ACHD) individuals have increased risk of noncardiac comorbidities including cancer and infections. Whether they are at increased risk of autoimmunity is unknown. OBJECTIVES: The purpose of this study was to understand the association of ACHD and risk for autoimmunity. METHODS: A case-control study was performed using All of Us, a nationwide biomedical database. In 2024, we queried autoimmune conditions and ACHD individuals ≥18 year of age with congenital heart disease (CHD) diagnoses determined using SNOMED, International Classification of Diseases (ICD)-9-CM, and ICD-100-CM classifications. ACHD individuals were matched 1:20 to controls for age, race, sex, smoking, and obesity. We examined subcategories of mild/moderate/severe CHD. RESULTS: Among 287,012 participants, 2941 ACHD (1.02%) individuals (mean age 62 years, 55.3% female) were identified. ACHD individuals had increased odds of autoimmunity (OR: 1.95; P < 0.05), both systemic (OR: 1.94; P < 0.05) and organ specific (OR: 1.54; P < 0.05) conditions. Sixty-nine percent had sufficient detail to be classified into mild/moderate/severe CHD; severe group was smaller and younger (n = 162, mean age = 47.6 years) compared to mild (n = 1169, mean age = 63.8) and moderate (n = 703, mean age = 61.8 years) groups. Mild and moderate CHD were associated with autoimmunity (OR: 1.93; P < 0.05 and OR: 1.56; P < 0.05, respectively). The entire severe CHD group did not show an increase in odds of autoimmunity (OR: 1.45; P = 0.168). When the groups were subdivided by age, severe ACHD individuals ≥60 years did show increased odds of autoimmunity (OR: 2.71; P < 0.05). CONCLUSIONS: ACHD individuals showed a nearly 2-fold increased odds of developing autoimmune conditions, both for systemic and organ-specific autoimmunity, which persisted among those with simple/moderate CHD. In an age-related analysis, the severe CHD group ≥60 years has a similar risk.