Abstract

BACKGROUND: Bicuspid aortic valve (BAV) is the most common congenital cardiac defect in the adult population, with a prevalence of 0.5%-2%. It is well recognized that aortic stenosis (AS), aortic regurgitation (AR) and aertopathy may develop by the fifth or sixth decade of life. There is a paucity of autopsy studies evaluating the hearts of subjects with BAV. The aim of this study is to ascertain the role of BAV in cases of sudden cardiac death. METHODS: A database of 6325 whole hearts referred to a specialist cardiac pathology center between 2004 and 2021 was reviewed to identify a subgroup of 91 subjects with a BAV reported. All cases had a negative full body autopsy and toxicology before being referred and subsequently underwent detailed cardiac evaluation including histological analysis by expert cardiac pathologists. RESULTS: The mean age of death was 37 ± 16 years (84% male). Death was attributed to aortic valve or aortic disease in 57% (n = 52) of cases; AS 30% (n = 27), endocarditis 11% (n = 10), aortic dissection (AD) 9% (n = 8) and AR 8% (n = 7). In the remaining 43% of cases, BAV was an incidental finding. CONCLUSION: The majority of deaths in young individuals with BAV were attributed to complications related to the aortic valve or aorta indicating that BAV is not a benign condition. When a BAV is identified, individuals should be appropriately follow-up with imaging to inform the optimal timing of intervention before a complication develops that may predispose the individual to a premature death.