The presentation and progression of bicuspid aortic valve disease in the modern era

Introduction Bicuspid aortic valve (BAV) is the most common valvular anomaly, affecting approximately 1-2% of the population. The natural history of BAV involves a high lifetime morbidity burden, with significant progression to valvular dysfunction, both stenosis and regurgitation. Despite these risks, contemporary management strategies, including regular surveillance and timely intervention, have improved outcomes and the natural course of the disease. Methods We conducted a retrospective cohort study at a tertiary medical center, analyzing sequential echocardiograms of patients with a bicuspid aortic valve. Patients were stratified by aortic stenosis severity and aortic regurgitation severity at presentation and followed longitudinally to assess condition progression, morbidities, and subsequent valvular interventions. Results Our study identified 1,733 BAV patients who underwent 18,601 echocardiograms from 1995 to 2024. The mean age at presentation was 50 years (interquartile range [IQR], 36 to 63 years), and 62% were male. At baseline, 1,288 patients had none/trivial aortic stenosis (AS), 203 (11.7%) had mild AS, 144 (8.3%) had moderate AS, and 98 (5.7%) had severe AS; 929 patients had none/trivial aortic regurgitation (AR), 576 (33.2%) had mild AR, 180 (10.4%) had moderate AR, and 48 (2.8%) presented with severe AR. Over a median follow-up of 12.4 years (IQR, 5.53–18.94), 44% progressed in AS severity, and 46% progressed in AR severity. Aortic valve intervention was required in 28% of patients, with 94.7% undergoing surgical aortic valve intervention and 5.3% being treated with transcatheter aortic valve implantation. Infective endocarditis occurred in 1.6% of the patients. Conclusions Patients with bicuspid aortic valve demonstrate a heterogeneous course, with a substantial proportion progressing to moderate or severe stenosis or regurgitation and ultimately requiring valve interventions. These findings emphasize the importance of individualized risk stratification, structured periodic surveillance, and timely intervention in this population. Figure 1 - Rates of valvular intervention by severity of aortic stenosis at presentation Figure 2- Rates of valvular intervention by severity of aortic regurgitation at presentationFigure 1 -AS Figure 2 -AR