Mechanisms of Dysfunction and Prognostic Markers in Bicuspid Aortic Valve

Background: The most common complication of the bicuspid aortic valve (BAV) is aortic valve dysfunction, but it is difficult to predictwhich patients will develop aortic stenosis (AS) or significant aortic regurgitation (AoR) (moderate/severe).Objectives: The aim of this work was to analyze the progression and the variables associated with the development of AS and significantAoR in adults with BAV.Methods: Consecutive patients with BAV were studied between 2009 and 2017. The progression of their aortic valve dysfunctionwas analyzed and in the group without baseline dysfunction, significant predictors of AoR and AS were identified through univariateand multivariate analysis.Results: Two hundred and forty-three patients (mean age 43±14.9 years, 73.2% men) were included in the study. The majority(n=194, 79.8%) with type I and raphe BAV (n=179, 73.6%). In the baseline echocardiogram, 111 patients presented mild (45.6%);49, moderate (20.1%); and 10, severe (4.1%) AoR. Baseline AS was less frequent: 20 subjects had moderate (8.2%) and 12, severe(4.9%) AS.Two patients died and 20 valve surgeries (8.2%) were performed in 4.7±1.7 follow-up years. Patients with significant baselinevalve dysfunction presented a higher rate of progression requiring valve surgery (p<0.0001). There were 39 new cases (17.2%) ofsignificant AoR or AS at follow-up. Aortic valve prolapse (p<0.001) and male sex (p<0.04) were associated with the development ofsignificant AoR (p<0.001). Baseline calcification score was associated with significant AS (p<0.02).Conclusions: A high proportion of patients with BAV and significant baseline aortic valve dysfunction required short-term surgery.Clinical and morphological characteristics associated with the development of significant aortic valve dysfunction were identified.