The team “Genetics and Development of Cardiac Defects” publishes in collaboration with the Department of Cardiology of “La Timone” Hospital the first study on a French cohort of patients with bicuspid aortic valve (BAV). Our project is designed to collect clinical and genetic data on consecutive patients prospectively admitted with a definite diagnosis of BAV. This study describes the clinical and echocardiographic spectrum of 223 consecutive BAV patients as they present in a tertiary care center and to determine factors associated with each type of valve degeneration. In this study we confirm high prevalence of valve dysfunction at first diagnosis of BAV in a referred population. The degenerative process differs according to type of dysfunction and is mainly dependent on age and gender.