Abstract

BackgroundRisk assessment in the expanding population of adult patients with congenital heart disease (ACHD) is of high importance. AimTo report clinical characteristics, complications, mortality rate and causes of death in a single-centre ACHD cohort. MethodsData were prospectively collected from an ACHD outpatient clinic from January 2012 until December 2019. ResultsOf 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]. ConclusionHF 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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