Characteristics and long-term outcomes of patients with bicuspid aortic valve results from a comprehensive aortic valve registry

Abstract Introduction Bicuspid aortic valve (BAV) is the most common congenital heart valve disease, presenting across a broad spectrum that ranges from non-dysfunctional valves to severe aortic stenosis (AS) and/or aortic regurgitation (AR). However, long-term outcome in a large cohort remains scarce. Objective This study aims to characterize the bicuspid aortic valve (BAV) population and evaluate outcomes in a large, single-center cohort of consecutive patients diagnosed with BAV between 1995 and 2020. Methods A retrospective cohort of consecutive patients diagnosed with BAV using echocardiography was included. Results A total of 2,188 patients (49 ± 17.1 years, male 71.6%) were included in the study. Ischemic heart disease was observed in 12.8% of patients, while 8% had a history of cerebrovascular events. AS was identified in 14.7% of patients, AR in 7.5%, and combined severe AS and AR in 1.3%. During a median follow-up time of 12 (interquartile range 10, range 0-29) years, the overall mortality rate was 20.8%, with a mean age at death of 80.6 ± 14.3 years. A total of 19.6% of patients underwent either surgical or transcatheter aortic valve interventions (TAVI). Among them, 16.8% underwent surgical procedures, including bioprosthetic valve replacement (11.2%), mechanical valve replacement (5.6%), aortic valve repair or Ross procedure (0.8%), and TAVI (1.7%),in addition, 134 (6.1%) patients also underwent aortic surgery . The incidence of infective endocarditis in the cohort was 3.6%. Conclusions BAV Registry highlights a high burden of aortic valve disease requiring surgical or transcatheter intervention , emphasizing the importance of early diagnosis.