Background The aim of this study was to evaluate the longer-term results of bicuspid aortic valve (BAV) repair with or without aortic root replacement. Methods From 1999 to 2017, 142 patients with or without aortic root dilatation who underwent repair of a regurgitant BAV were included in the study. Ninety-four patients underwent isolated BAV repair (Group 1; mean age 45±14 years) and 48 patients underwent valve-sparing aortic root replacement plus BAV repair (aortic valve reimplantation – Group 2; mean age 49±13 years. Median follow-up time was 5.9 years (range 0.5-15) in Group 1 and 3 years (range 0.5-16) in Group 2, respectively. Results In-hospital mortality was 1% in group 1, and 2% in Group 2 (p=0.6). The 5- and 10-year survival was 93±2.9% and 81±5.8% in Group 1 and 96±3.1% and 96±3.1% in Group 2, respectively (p=0.31). Eleven patients of Group 1 (1.7% /patient-year) and 5 patients of Group 2 (2.2%/patient-year) underwent reoperation of the aortic valve (p=0.5). The 5- and 10-year freedom from reoperation were 93.0±2.1% and 77.1±7.1% in Group 1 and 93.0±5.0% and 76.7±9.6% in Group 2 (p=0.83), respectively. At latest follow-up only 2 patients of Group 1 and 1 patient of Group 2 had AR=2° (p=0.7). The cumulative linearized incidence of all valve-related complications (bleeding, stroke, endocarditis, reoperation) was 2.9%/patient-year in Group 1 and 4%/patient-year in Group 2, respectively (p=0.6). Conclusions Isolated BAV repair and combined aortic valve reimplantation plus BAV repair provide good clinical longer-term outcomes with relatively low reoperation rate and durable valve function.