Long-term survival in adults with congenital heart disease: a nationwide, register-based cohort study

Abstract

Abstract Background The survival of children with congenital heart disease has increased exponentially over the last decades, with 97% currently reaching adulthood. However, the total impact of advanced treatment on mortality and morbidity in adult survivors in relation to the general population is less well described. Purpose We aimed to investigate long term outcomes of adults with congenital heart disease (ACHD) in Sweden, from the age of 18 years through middle-age. Methods Data from the Swedish National Patient Register and Cause of Death Register were linked to identify all ACHD patients born from 1950 to 1999 and alive at the age of 18 years. Each ACHD patient was matched by birth year and sex with more than ten controls without congenital heart malformations from the Total Population Register in Sweden, with follow-up time for cases and controls until December 2017. Results Altogether 37,278 ACHD patients and 412,799 matched controls were included in the study. The mean follow-up was 19.2 (± 13.6 years). The risk of death after the age of 18 years was 3.2 times higher (95%, confidence interval (CI) 3.04–3.37) among ACHD patients compared to controls. At the age of 68 years, more than 75% of ACHD patients were still alive. The risk of death was significantly higher also for the more benign lesions i.e. atrial septal defects, ventricular septal defects and coarctation of the aorta. Starting in the mid 1970ies, the risk of death declined over time and for those born after 1975 the risk decreased steadily with each successive birth year. More patients with ACHD developed atrial fibrillation, heart failure, non-fatal myocardial infarction, non-fatal stroke or died in comparison to controls, adjusted hazard ratio for any of these ranged between 20.58 (95%, CI 18.74–22.60) for patients with complex disease and 9.89 (95%, CI 9.53–10.27) for patients with mild or moderate disease. Conclusions In this large, nationwide, register-based cohort study of patients with ACHD surviving until the age of 18 years, the risk of mortality up to the age of 68 years was more than three times higher compared to matched controls. A notable decline in the risk of death was found in patients with ACHD born after 1970. Despite this substantially high relative mortality risk during adulthood, at least 75% of ACHD patients will become sexagenerians. Funding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Swedish state under an agreement between the Swedish Government nad county Councils (ALF)