Abstract BACKGROUND AND OBJECTIVES: Bicuspid aortic valve (BAV) disease is the most common congenital cardiac malformation. The aim of the present article is to determine clinical and echocardiographic prognostic factors and provide a predictive model of outcome of a large cohort of patients with BAV. METHODS: We retrospectively enrolled 337 patients consecutively assessed for echocardiography at our Cardiology Department from 1993 to 2014. We considered aortic valve replacement, aortic surgery and cardiovascular death as a clinical combined end-point. Predictors of outcome were determined by Cox regression. RESULTS: Mean age was 29.2 ± 19.8 years, median 27.1 years. A total of 38.4% patients presented a history of hypertension. Mean duration of follow-up was 8.4 ± 6.1 years, range 0-21 years. A total of 73 patients underwent aortic valve replacement and/or aortic surgery during follow-up. Age at surgery was 45.2 ± 15.6 years. Seven patients died because of cardiovascular causes. At multivariate analysis, baseline clinical predictors were history of hypertension [hazard ratio (HR) 2.289, 95% confidence interval (CI) 1.350-3.881, P = 0.002], larger ascending aortic diameter (HR 2.537, 95% CI 1.888-3.410, P