Background and Aims: Bicuspid aortic valve (BAV) related aortopathy has been a controversial issue in the past few years. Most of the researches focused on BAV with dilated ascending aorta after aortic valve replacement (AVR), but there were limited documents of patients with the normal-sized proximal aorta. We retrospectively analyzed the clinical data of patients undergoing AVR in our institution and evaluated the progression of the unreplaced ascending aorta in a relatively long term follow-up. Methods: In our institution, 165 patients were consecutively recruited from July 2004 to December 2017. Detailed perioperative information and follow-up data were comprehensively collected and quantitatively analyzed. Results: 48 patients (29.1%) had BAV, while TAV was found in 117 patients. A significant difference was observed in diameters of ascending aorta at baseline between BAV and TAV group (37.5 ±4.2mm vs 35.1 ±4.4mm; p=0.001). The overall survival rates were 89% and 95.8% at 10 years postoperatively in BAV versus TAV group (Plog rank=0.138). Only 1 patient suffered an aortic dissection and underwent proximal aortic surgery. No difference in the progression of ascending aorta (0.8±4.7mm vs 0.6±3.5mm, p=0.821) was observed. The diameter of ascending aorta at baseline was a significant predictor of progression in ascending aorta. Conclusions: BAV patients with a normal-sized ascending aorta have a considerable low incidence of late adverse aortic events after AVR. Meanwhile, the progression of the unreplaced ascending aorta in BAV patients is not different from that in TAV patients.