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The bicuspid aortic valve, a kind of heart disease that comes from parents, has been paid attention around the world. Although most bicuspid aortic valve (BAV) patients will suffer from some complications including aortic stenosis, aortic regurgitation, endocarditis, and heart dysfunction in the late stage of the disease, there is none symptom in the childhood, which restrains us to diagnose and treatment in the onset phase of BAV. Hemodynamic abnormalities induced by the malformations of the valves in BAV patients for a long time will cause BAV-associated aortopathy: including progress aortic dilation, aneurysm, dissection and rupture, cardiac cyst and even sudden death. At present, preventive surgical intervention is the only effective method used in this situation and the diameter of the aorta is the primary reference criterion for surgery. And the treatment effects are always not satisfactory for patients and clinicians. Therefore, we need more methods to evaluate the progression of BAV and the surgery value and the appropriate intervention time by combining basic research with clinical treatment. In this review, advances in morphology, genetic, biomarkers, diagnosis and treatments are summarized, which expects to provide an update about BAV. It is our supreme expectations to provide some evidences for BAV early screening and diagnosis, and in our opinion, personalized surgical strategy is the trend of future BAV treatment.