The clinical characteristics and outcome of intracardiac thrombus and aortic valvular involvement in Behçet's disease: an analysis of 20 cases.

OBJECTIVES: To assess the clinical characteristics and outcome of patients with cardiac Behçet's disease(BD). METHODS: Medical charts of 20 cardiac BD patients admitted in Peking Union Medical College Hospital from June 1996 to June 2011 were systematically reviewed, including demographic data, clinical features, laboratory and histopathology findings and outcome. RESULTS: Patients age ranged 19~57 yrs[mean (35±10) yrs], included 17 males and 3 females. Six (30%) of them did not fulfill the ISG criteria at cardiac onset, and fourteen (70%) of them experienced heart failure. Echocardiography findings included intracardiac thrombus (n=7), and aortic valve involvement with left ventricular enlargement and severe aortic regurgitation (n=13). Eight patients underwent surgery before efficient immunosuppressant treatment, and five (62.5%) underwent re-operation due to recurrence of thrombus or valvular dehiscence and severe paravalvular leakage. Histopathology findings revealed predominantly inflammatory cells infiltration, thrombus and fibrous tissue formation. After initiation of prednisone plus immunosuppressant, patients were followed up for 6~42 months (mean 14.8±9.9 months), the intracardiac thrombus disappeared or decreased in size in five cases, remained stable after surgery in the other two cases, and the heart failure disappeared in all patients with aortic involvement. CONCLUSIONS: Cardiac BD affects males more than females, and is prone to delayed diagnosis because some patients do not have typical clinical manifestations at cardiac onset; Corticosteroids plus immunosuppressants reduce the thrombus and improve aortic regurgitation and heart failure in cardiac BD, whereas surgery alone does not lead to complete resolution.