Aortic valve disease in patients with Wegener's granulomatosis

Wegener's granulomatosis usually affects the upper and lower respiratory tract as well as the kidney. Cardiac involvement is rare, although electrocardiographic abnormalities, coronary artery vasculitis, cardiac arrhythmias, and myocardial infarction have been described. We report two cases of aortic valve disease associated with Wegener's granulomatosis that were progressive despite clinical remission of Wegener's disease in both patients. One patient has undergone successful valve replacement and the other is currently awaiting surgery. Aortic valve histopathology showed myxoid degeneration that was most likely due to previously active vasculitis affecting the vessels of the aortic wall and valvular necrosis with subsequent progressive degeneration of the cusps.