ObjectiveTo summarize the characteristics of bicuspid aortic valve (BAV) aortopathy and analyze the association between aortopathy and BAV phenotype and patterns of valvular dysfunction.MethodsClinical data of 191 patients who underwent the first aortic valve replacement in Fuwai Hospital from June 2017 to March 2018 were retrospectively analyzed, including 143 males and 48 females with an average age of 53.91±12.52 years. All patients underwent multidetector computed tomography (MDCT) and echocardiography before the operation, excluding patients with aortic coarctation. The BAV aortopathy phenotype was classified during operation. The characteristics of BAV aortopathy were analyzed by cluster and artificial analysis. BAV anatomic phenotype was divided into two types according to the direction of valve opening: BAV-AP and BAV-LR.ResultsFour distinct BAV aortopathy phenotypes were identified: a common type (n=70, 36.6%), with no dilation or mild dilation of aorta; a root type (n=24, 12.6%), with predominant dilatation of aortic sinus; an ascending aorta type (n=72, 37.7%), with predominant dilatation of ascending aorta; an arch type (n=25, 13.1%), with predominant dilatation of aortic arch dilatation. The root type was mainly in young patients, while the arch type was mainly in elderly patients (P<0.05). BAV-AP and aortic insufficiency were most prevalent in root type, while BAV-LR and aortic stenosis were most prevalent in arch type (P<0.05). There were 111 (58.1%) patients undergoing aortic surgery, and the coincidence rate of BAV aortopathy phenotype and aortic surgery was 80.6%.ConclusionAccording to the location of aortic dilation, BAV aortopathy can be divided into four types. There is an association between BAV aortopathy and valvular phenotype and dysfunction.