Abstract
A 73-year-old woman was admitted with shortness of breath on exertion. Echocardiography revealed a dilated sinus of Valsalva with aortic regurgitation. Cardiac catheterization and angiography showed an extracardiac extension of an aneurysm of the right coronary sinus of Valsalva, right coronary ostial stenosis and severe aortic regurgitation. An aortic root replacement was performed with a full root stentless valve, the Freestyle aortic bioprosthesis (Medtronic, Inc, Minneapolis, MN, USA) implantation combined with single coronary artery bypass grafting (CABG) to the right coronary artery (RCA). The pathological findings strongly suggested congenital microfibrillar disease.