Prevalence, clinical characterization, management and evolution of bicuspid aortic valve classified according to the 2021 International Consensus Statement in a tertiary care hospital.

Objectives: The 2021 International Consensus for the congenital bicuspid aortic valve (BAV) condition recognizes 3 morphologies of BAV (fused, two-sinus and partial-fusion) and 3 types of aortopathy (ascending, root and extended). The clinical impact of BAV phenotyping on aortopathy evolution has not been evaluated so far. The aims were to assess: (i) prevalence of BAV phenotypes; (ii) frequency of BAV-related aortic valve dysfunction and aortopathy; and (iii) inter-phenotypic differences in aortopathy progression in a real-world population.

Methods: This was an observational cohort study on patients with BAV 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.

Results: Three hundred and two patients were evaluated: 245 (81.1%) had fused, 41 (13.6%) two-sinus and 16 (5.3%) partial fusion BAV. 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 3 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 two-sinus type, at a median follow-up of 2 years.

Conclusions: Fused type represented the BAV phenotype with the highest frequency and the most significant association with aortopathy. In terms of aortopathic progression, the mid-term growth rate of the thoracic aorta was more significant in the non-interventional fused BAVs.