Pervious studies indicated that the prevalence of bicuspid aortic valve（BAV） might be higher in China. There is a familial predisposition to the development of BAV. The study analyzed epidemiological data on selected BAV and established a BAV cohort to observe disease progression and treatment outcomes.

There is no recognized comprehensive epidemiological data on congenital BAV in China, although findings of BAV is common in clinical practice. In the course of the Venus-A registry clinical study, which was the first Aortic Valve Replacement (TAVR) trial in China, the investigators concluded about 40% of the final enrollment of BAV patients. Since then, an increasing number of scholars have figured out that the prevalence of BAV may be much higher in China than abroad. This study was designed to investigate Gene backgrounds, pathological progress, imaging characteristics of young health BAV participants and their relationships in long-term follow-up. These findings may help to identify high risk individuals with specific gene expression or other features in clinical observation.

Inclusion Criteria:

• The patients were aged 14-60 years;
• Definitive diagnosis of BAV by imaging (echocardiography, aortic CTA, cardiac magnetic resonance, etc.)
• Patients were informed about the nature of the study and agreed to participate in all the terms of the study and signed an informed consent form approved by the ethics committee.

Exclusion Criteria:

• Poor patient compliance and inability to complete follow-up visits as required;
• Rheumatic heart valve disease, other organic valve disease.
• Hyperthyroidism, severe anemia, and other factors that significantly affect hemodynamics.
• Other circumstances that the researcher believes should be excluded.

This is single-center cohort study. Patients diagnosed with BAV were enrolled in Ruijin Hospital.