Objective To examine the results of root replacement with aortic valve sparing in patients with bicuspid aortic valve (BAV) or severe aortic regurgitation (AR) Methods Between 2000 and 2009, 102 patients (mean age 47 +/- 17 5 years) underwent aortic valve sparing procedures for ascending aortic aneurysm or dissection Patients were assigned to three different groups according to the aortic valve pathology BAV (n = 11), tricuspid aortic valve (TAV) with AR less than severe (n = 51), and TAV with severe AR (n = 40) Remodelling of the aortic root was performed in 28 (27 5%) patients, reimplantation of the aortic valve in 74 (72 5%) and a concomitant cusp repair in 30 (29 4%) All patients were prospectively studied with annual clinical assessment and echocardiography Results The overall actuarial 5 years survival was 97 8 +/- 1 5% without differences between the groups Actuarial 5 years freedom from aortic valve related re operation was 92 2 +/- 3 2% in all patients, 100% in patients with a BAV, 98 +/- 1 9% in patients with a TAV and AR less than severe, and 82 7 +/- 7 5% in patients with a TAV and severe AR ( p = 0 07) The overall actuarial freedom from AR, which was more than mild at 5 years was 73 3 +/- 7 7%, being significantly (log rank test p = 0 005) lower for patients presenting with TAV and severe AR (49 9 +/- 16 4%) Conclusions The outcome in terms of survival is excellent for all patients after aortic valve sparing operations There is no significant difference in terms of re operation between patients, who presented with BAV or TAV Re operation rate are higher for patients who presented with severe AR but these rates do not reach statistical significance Hence, root replacement with aortic valve sparing should be offered even in the presence of a BAV or severe AR (C) 2010 European Association for Cardio Thoracic Surgery Published by Elsevier B V All rights reserved