Clinical and echocardiographic predictors of long-term outcome of a large cohort of patients with bicuspid aortic valve

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 29.219.8 years, median 27.1 years. A total of 38.4% patients presented a history of hypertension. Mean duration of follow-up was 8.4 +/- 6.1years, 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<0.001), moderate-to-severe aortic regurgitation (HR 2.266, 95% CI 1.402-3.661, P=0.001) and moderate-to-severe aortic stenosis (HR 2.807, 95% CI 1.476-5.338, P=0.002). A predictive model was created by integrating these four independent covariates. It allows the calculation of calculate a risk score for each patient, which helps better tailor appropriate treatment in BAV patients. Conclusion At enrolment, history of hypertension, a wider aortic diameter, moderate-to-severe aortic regurgitation and aortic stenosis were independently correlated to combined end-point. Long-term follow-up showed low cardiovascular mortality (2.1%) and a high prevalence of cardiac surgery (21.6%).