Objective To investigate the value of echocardiography in follow-up management and prognosis analysis of patients with bicuspid aortic valve (BAV). Methods This study was a single-center retrospective clinical study. Patients diagnosed as "bicuspid aortic valve malformation" by echocardiography in the First Affiliated Hospital of Nanjing Medical University with complete clinical data and follow-up data, from January 2013 to December 2019, were included to compare the proportion of patients with different follow-up duration, and to compare the changes of valve function and aortic function between the initial diagnosis and the last follow-up. Furthermore, gender, age, morphological type, degree of aortic stenosis, degree of regurgitation, and inner diameter of ascending aorta were included in the regression analysis to find the risk factors affecting the prognosis of BAV. Results A total of 1154 patients with BAV were screened out, and 243 patients (21.06%) with complete follow-up data were enrolled. With longer follow-up, patients number became less, aortic valve dysfunction worsened and the inner diameter of ascending aorta widened gradually. Age, degree of AS and degree of AR at initial diagnosis were related risk factors for BAV requiring surgical intervention. Age was found to be an independent risk factor for the prognosis of the disease by drawing the subject curve and survival curve. The cut-off value of age was 36 years, and the sensitivity and specificity were 94.29% and 39.49%, respectively. The probability of no surgical event within 5 years in group of age less than 36 was 92.39%, while in the group of age more than 36, the probability was only 52.20%. Conclusion The follow-up rate of BAV patients in China is low. Valvular dysfunction and ascending aorta widening induced by BAV showed a time cumulative effect. For BAV group over 36 years old, healthy education should be strengthened, and echocardiography examination was suggested every year continuously to guide treatment.