Abstract
A 72-year-old woman was referred to our institution with decompensated congestive heart failure owing to subacute severe aortic regurgitation and mitral regurgitation. Her blood sample tested positive for myeloperoxidase anti-neutrophil cytoplasmic antibody (ANCA). Cardiac computed tomography revealed abnormal thickening and shortening of the aortic valvar leaflets as well as wall thickening of the sinuses of Valsalva. Based on the diagnosis of ANCA-associated vasculitis, predominantly involving the aortic root, prednisolone administration was initiated, which failed to improve the valvar dysfunction. The patient underwent aortic root replacement and mitral annuloplasty. Histopathology confirmed severe inflammation involving both the aortic valvar sinuses and leaflets.