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 29.2 ± 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 < 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%).