Cardiovascular Disease Burden in Adults with Congenital Heart Disease with Down Syndrome: Everything Worse or Better?

Background: Aging adults with congenital heart disease (ACHD) are prone to develop acquired cardiovascular disease. In Down syndrome (DS), presumed higher metabolic risk factors, congenital kidney disease, and impaired immunology causing chronic inflammation might modify cardiovascular and all-cause morbidity and mortality. Presently, it is unclear, whether DS is burdened by an elevated cardiovascular risk (CVR).

Methods: Differences in classical CVR factors and organ dysfunction were analyzed in 114 patients (38 DS; 76 age, sex, and heart defect-matched controls; age 36 ± 6.8 vs. 36.3 ± 10.29 years). Based on these findings, we evaluated the cardiovascular morbidity and mortality risk in various established risk scores (LIVE CVD model, MAGGIC, PREVENT, Reynolds Risk Index).

Results: DS had a higher BMI (29.01 ± 5.48 vs. 25.47 ± 4.61 kg/m²; p < 0.01), but significantly lower systolic blood pressure (p = 0.008) and less active smokers (p = 0.007). HBA1c and cholesterol levels were comparable. DS showed significantly higher sCRP (p = 0.014) and more severe renal impairment (p = 0.001). In DS, the ESC LIVE CVD Model calculated a significantly lower cardiovascular mortality risk (10 years: 0.6 ± 0.49% vs. 1.2 ± 2.02%, p = 0.0015; Lifetime: 21.89 ± 5.37% vs. 25.4 ± 9.14%, p = 0.012). The risk assessment of all other analyzed scores was similar.

Conclusion: ACHD with DS exhibited both, lower (less smoking, lower blood pressure) and higher CVR factors (elevated sCRP and BMI, worse renal function). Only the LIVE CVD model, which excluded sCRP and renal function, calculated a lower CVR in DS. Disparities in parameter selection and their weighting may explain diverging results. Our findings illustrate that the handicapped, usually excluded from studies, might profit from surveys focusing on their distinctive conditions.

Publication History

Article published online:
11 February 2025