BACKGROUND: Unicuspid aortic valve is an important subset of bicuspid aortic valve, and knowledge regarding its aortopathy pattern and surgical outcomes is limited. Our objectives were to characterize unicuspid aortic valve patients, associated aortopathy, and surgical outcomes. METHODS: From January 1990 to May 2013, 149 adult unicuspid aortic valve patients underwent aortic valve replacement or repair for aortic stenosis (n = 13), regurgitation (n = 13), or both (n = 123), and in 91 (61%) the aortic valve operation was combined with aortic repair. Data were obtained from the Cardiovascular Information Registry and medical record review. Three-dimensional imaging analysis was performed from preoperative computed tomography and magnetic resonance imaging scans. The Kaplan-Meier method was used for survival analysis. RESULTS: Patients had a mean maximum aortic diameter of 44 ± 8 mm and variably involved the aortic root, ascending, or arch, or both. Patients with valve operations alone were more likely to be hypertensive (p = 0.01) and to have severe aortic stenosis (p = 0.07) than those who underwent concurrent aortic operations. There were no operative deaths, strokes, or myocardial infarctions. Patients undergoing aortic repair had better long-term survival. Estimated survival at 1, 5, and 10 years was 100%, 100%, and 100% after combined operations and was 100%, 88%, and 88% after valve operations alone (p = 0.01). CONCLUSIONS: Patients with a dysfunctional unicuspid aortic valve frequently present with an ascending aneurysm that requires repair. Combined aortic valve operations and aortic repair was associated with significantly better long-term survival than a valve operation alone. Further study of this association may direct decisions about timing of surgical intervention.