| Summary: | Background: Risk assessment in the expanding population of adult patients with congenital heart disease (ACHD) is of high importance. Aim: To report clinical characteristics, complications, mortality rate and causes of death in a single-centre ACHD cohort. Methods: Data were prospectively collected from an ACHD outpatient clinic from January 2012 until December 2019. Results: Of 356 patients (median age 34 years, 52.5% female), 116 (32.6%) were classified as simple, 182 (51.1%) as moderate and 58 (16.3%) as complex ACHD. At baseline assessment, more than half (n = 184) were symptomatic, while 69 patients (19.4%) had a history of arrhythmia and 21 (5.9%) a history of heart failure (HF) hospitalisation. Over a median follow-up of 4 years (1–7 years), 29 (8.1%) patients died, mainly due to HF. In multivariate Cox regression analysis, age [hazard ratio (HR) 1.03 (confidence interval (CI) 1.00–1.05), p = 0.034], Bethesda classification [HR 1.77 (CI 1.05–3.02), p = 0.034], NYHA class III/IV [HR 3.28 (CI 1.19–9.04), p = 0.022] and history of HF hospitalisation [HR 3.79 (CI 1.27–11.30), p = 0.017] predicted all-cause death. A total of 16.3% of patients developed complications, which were independently predicted by age [HR 1.03 (CI 1.01–1.05), p = 0.004], history of surgical procedure [HR 1.45 (CI 1.12–1.86), p = 0.004] and history of HF hospitalisation [HR 3.38 (CI 1.56–7.29), p = 0.002]. Conclusion: HF was the leading cause of death in this ACHD cohort and HF hospitalisation was a predictor of morbidity and mortality. These findings highlight the need for prevention of HF hospital admissions and strategies to improve postdischarge outcomes.
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