No data are available on the natural history and outcomes of middle-aged European patients with bicuspid aortic valve (BAV), as the only 2 large studies were conducted on young adults in Canada and north America. We aim to determine the frequency of cardiovascular events and to assess the survival relative to that of the general population in a large consecutive series of adults diagnosed with BAV in our echocardiographic laboratory according to the presence or the absence of a valvular and/or vascular surgical indication at the time of diagnosis.

Between 2005 and 2017, 350 patients without surgical indication (medical group, mean age 53 years, 71% of men) and 191 patients with surgical indication (surgical group, mean age 59, 71% of men) at baseline have been included. Median follow-up was 80 [32–115] months.

In the medical group, 34 deaths (9.7%) were recorded (10 cardiovascular-related). The 5-years and 10-years survival rate were respectively 93±1% and 88±2% with a relative survival of BAV patients compared to the age-and-sex-matched population of 98.8%. Nine patients (2.6%) were diagnosed with infective endocarditis (IE) and no aortic dissection occurred. Aortic valve and/or ascending aorta surgery was performed in 102 patients (29%) during follow-up. In the surgical group, 12 deaths (6.3%) were recorded (5 cardiovascular-related). The 5-years and 10-years survival rate were respectively 97±1% and 90±3% with a relative survival of BAV patients compared to the age-and-sex-matched population of 99.2%. Five patients (2.6%) were diagnosed with IE and no aortic dissection occurred.

In BAV, survival rates are not lower than for the general population. The rate of IE is low and no aortic dissection occurred in our study. Middle-aged adults with BAV have a high likelihood of surgery on the aortic valve and/or ascending aorta.