OBJECTIVES: Bicuspid aortic valve (BAV)-associated aortopathy has been proposed to progress after isolated aortic valve replacement (AVR) surgery, which has been traditionally used as an argument against a TAVR procedure in this clinical subset. Still, more than half of BAV patients have a normal-sized proximal aorta at the time of AVR surgery. We aimed to analyse the long-term risk of adverse aortic events after isolated conventional AVR surgery for BAV and normal-sized proximal aorta. METHODS: A total of 200 consecutive patients (mean age 56 ± 13 years, 73% men) with BAV disease and ascending aortic diameter of <40 mm underwent isolated conventional AVR from 1995 through 2008 and were identified from our institutional BAV database. Long-term follow-up data (a total of 1532 patient-years) were obtained for all hospital survivors. Composite adverse aortic/cardiovascular events were defined as the need for redo aortic surgery, the occurrence of aortic dissection/rupture, or sudden cardiac death during follow-up. RESULTS: A total of 25 (13%) patients died after a mean follow-up of 8.5 ± 5 years post-AVR surgery, which resulted in an overall survival rate of 75 ± 6 and 87 ± 7% at 15 years postoperatively, for BAV-aortic stenosis and BAV-aortic regurgitation, respectively (P = 0.6). An adverse aortic event occurred in 1 (0.5%) study patient, while 5 (2.5%) further patients suffered sudden cardiac death. Only 1 patient required redo aortic surgery. No documented aortic dissection/rupture occurred. Redo AVR surgery due to endocarditis or structural valve degeneration was performed in 6 (3%) patients. The rate of freedom from composite adverse events was 92 ± 5 and 95 ± 4% at 15 years, for the AS and AR subgroups, respectively (P = 0.7). CONCLUSIONS: BAV patients with aortic valve dysfunction and normal-sized ascending aorta are at considerably low risk of late adverse aortic events after isolated AVR.