Cardiac Behçet disease presenting as aortic valvulitis/aortitis or right heart inflammatory mass: a clinicopathologic study of 12 cases.

Behçet disease is an inflammatory disorder of unknown etiology showing diverse clinical presentations. Cardiac involvement is a critical problem that requires a timely diagnosis and management. However, clinicopathologic features have not been characterized clearly. Here, we present clinicopathologic characteristics of this uncommon disease. Patients included 8 males and 4 females, ranging from 24 to 52 years old. They were presented with abrupt heart failure and were mostly diagnosed as having cardiac Behçet disease later in the course. Upon echocardiography, 8 patients showed severe aortic regurgitation with redundant prolapsing aortic cusps and 4 patients showed irregular mass lesions in the right ventricular cavity. No one had both lesions. The aortic root was also involved with aortic valvulitis, showing severe mixed acute and chronic inflammation of various stages. There were frequent microabscess and extensive endothelial loss with fibrinous deposit. The right heart lesions showed similar histopathologic features. Four patients who initially underwent simple aortic valve replacement developed serious postoperative complications requiring reoperations. No serious complications developed after the treatment was changed to a replacement of aortic root with extensive debridement and concomitant immunosuppressive therapy. Cardiac Behçet disease is presented as aortic valvulitis/aortitis or inflammatory mass lesion. Characteristic echocardiographic and pathologic findings seem to be helpful for the timely diagnosis of this critical disease.