Prevalence, clinical characterization, management, and evolution of bicuspid aortic valve classified according to the 2021 international consensus statement in a tertiary care hospital

The 2021 International Consensus for the congenital bicuspid aortic valve condition recognizes 3 morphologies of bicuspid aortic valve (fused, 2-sinus and partial-fusion) and 3 types of aortopathy (ascending, root and extended). The clinical impact of bicuspid aortic valve phenotyping on aortopathy evolution has not been evaluated so far.The aims were to assess: 1) prevalence of bicuspid aortic valve phenotypes; 2) frequency of bicuspid aortic valve-related aortic valve dysfunction and aortopathy; 3) inter-phenotypic differences in aortopathy progression in a real-world population. This was an observational cohort study on patients with bicuspid aortic valve referred to our tertiary hospital between January 2018 and November 2022 to undergo a comprehensive transthoracic echocardiography. Baseline clinical, ultrasonographic and computed tomographic data were evaluated; even echocardiographic progression of aortic dilatation was assessed. Three hundred and two patients were evaluated: 245 (81.1%) had fused, 41 (13.6%) 2-sinus and 16 (5.3%) partial fusion bicuspid aortic valve. Aortopathy was documented in 101 (33.6%) cases and it was prevalent among patients with the fused type. The prevalence of aortic valve dysfunction was instead similar among the three groups. Two hundred and twelve patients underwent invasive management of clinically relevant aortic valve or aortic disease. Non-operated fused type presented the highest progression rate of aortic dilatation, whilst, among the interventional subpopulation, a more pronounced evolution was observed in the 2-sinus type, at a median follow-up of 2 years. Fused type represented the bicuspid aortic valve phenotype with the highest frequency and the most significant association with aortopathy. In terms of aorthopatic progression, the mid-term growth rate of the thoracic aorta was more significant in the non-interventional fused BAVs.