The patient presented with a history of recurrent aphthous stomatitis, genital ulceration, and a family history of positive for collagen disease. Echocardiography and retrograde aortography revealed aneurysm formation of the sinus of Valsalva, and dilatation of the aortic valve annulus with severe aortic regurgitation. On diagnosis of an aneurysm of the sinus of Valsalva and aortic regurgitation associated with Behçet's disease, aortic root replacement with the modified Bentall technique was successfully performed.
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