Background: We reviewed the experience of An Zhen and Fu Wai Hospital for congenital sinus of Valsalva aneurysm (SVA) to determine risk factors for aortic valve replacement (AVR) and postoperative progression of aortic regurgitation (AR). Methods: Over a 7-year period, 255 patients underwent surgical repair of an SVA. Aneurysms originated from the right sinus and the noncoronary sinus in 212 patients (83.1%) and 38 patients (14.9%), respectively, and protruded into the right ventricle and right atrium in 171 patients (67.1%) and 80 patients (31.4%), respectively. AR presented in 142 patients (55.7%), 60 patients underwent AVR, and 13 patients underwent aortic valvuloplasty (3 patients eventually received AVR for valvuloplasty failure). Results: All patients survived the operation. Late death occurred in 2 patients (0.8%), and 2 patients (0.8%) experienced anticoagulation-related complications. Logistic regression analysis revealed that infective endocarditis, the cardiothoracic ratio, and a nonruptured SVA were risk factors for AVR. Late follow-up of 150 patients by echocardiographic assessment revealed that AR improved in 17 patients and worsened in 20 patients. Cox regression analysis revealed AR at discharge to be an independent risk factor for AR aggravation at late follow-up. Conclusions: SVA can be repaired with low mortality and excellent long-term results. AR at discharge is an important factor in determining AR aggravation at late follow-up after the operation. We recommend early diagnosis and aggressive treatment for SVA.