Background: Unicuspid and bicuspid aortic valve (BAV) are congenital cardiac anomalies associated with valvular dysfunction and aortopathies occurring at a young age.

Objectives: To evaluate our experience with aortic valve repair (AVr) in patients with bicuspid or unicuspid aortic valves.

Methods: Eighty patients with BAV or unicuspid aortic valve (UAV) underwent AVr. Mean patient age was 42 ± 14 years and 94% were male. Surgical technique included: aortic root replacement with or without cusp repair in 43 patients (53%), replacement of the ascending aorta at the height of the sino-tubular junction with or without cusp repair in 15 patients (19%), and isolated cusp repair in 22 patients (28%).

Results: The anatomical structure of the aortic valve was bicuspid in 68 (85%) and unicuspid in 12 patients (15%). Survival rate was 100% at 5 years of follow-up. Eleven patients (13.7%) underwent reoperation, 8 of whom presented with recurrent symptomatic aortic insufficiency (AI). Late echocardiography in the remaining 69 patients revealed mild AI in 63 patients, moderate recurrent AI in 4, and severe recurrent AI in 2. Relief from recurrent severe AI or reoperations was significantly lower in patients who underwent cusp repair compared with those who did not (P = 0.05). Furthermore, the use of pericardial patch augmentation for the repair was a predictor for recurrence (P = 0.05).

Conclusions: AVr in patients with BAV or UAV is a safe procedure with low morbidity and mortality rates. The use of a pericardial patch augmentation was associated with higher repair failure.