Abstract Introduction Adults with congenital heart disease (ACHD) represent a growing and heterogeneous population with increasing longevity and evolving risk profiles. As survival improves, many ACHD patients develop a range of complications and comorbidities, including acquired cardiovascular risk factors. These factors may influence long-term outcomes, particularly late mortality. Purpose To investigate the association between acquired cardiovascular risk factors and late mortality in a large cohort of ACHD patients, with a focus on how modifiable factors contribute to adverse outcomes and death. Methods We performed a retrospective cohort study of 9,824 ACHD patients from our tertiary ACHD centre, with all-cause mortality as the primary outcome. Adopting a multi-perspective approach, encompassing demographic, clinical, metabolic, psychological, and socioeconomic variables, we conducted univariable analyses to identify key risk factors associated with mortality. Multivariable Cox proportional hazards analysis was used to determine independent predictors of mortality. Results Overall, 9,824 patients with a mean age of 39.0 years were included (50.3% male). Underlying diagnoses covered the entire sprectrum of congenital diagnoses (NYHA class 1 (75.6%), class 2 (18.8%), class 3 (5.3%), and class 4 (0.3%)). During a median follow-up time of 10.0 years (interquartile range, 4.9-14.4; corresponding to a total of 98748 patient-years), 1,369 patients died. In the univariable analysis, both non-modifiable and modifiable risk factors were identified as significant predictors of death. The multivariable analysis revealed that older age (HR 1.07 per year, p0.001), male sex (HR 1.26, p=0.020), higher lesion complexity (HR 2.11, p0.001), and lower income (HR 0.86, p=0.001) were significant non-modifiable factors worsening late mortality. Among the modifiable factors, sleep apnoea (HR 2.32, p0.001), ischemic heart or cerebral disease (HR 1.62, p0.001), depression (HR 1.38, p=0.008), and smoking (ex-smoker: HR 1.33, p=0.025, current-smoker: HR 1.36, p = 0.026) were significantly associated with increased risk of death. Subgroup analyses by smoking status revealed that among ever-smokers (former and current), diabetes emerged as an additional risk factor (HR 1.86, p=0.012), whereas it was not significant among never-smokers, suggesting that avoiding smoking may help mitigate the adverse metabolic risk in ACHD. Conclusion Our study underscores the necessity of taking proactive steps to manage modifiable factors, particularly sleep apnea, depression, smoking, and those related to preventing ischemic cardiovascular disease, to achieve better long-term outcomes in ACHD. In addition to conventional ACHD-specific treatments, it is essential to adopt a comprehensive preventive approach that emphasises early detection and management—or even prevention—of these factors in order to reduce mortality.Summary Illustration
Umei et al. (Sat,) studied this question.