BACKGROUND AND OBJECTIVES: Despite the sporadic case reports on the cardiac involvement of Behcet's disease, any systemic investigation has not been reported on. We sought to investigate the frequency and clinical characteristics of the patients with the cardiac manifestations of Behcet's disease. SUBJECTS AND METHODS: We retrospectively analyzed the clinical data of 217 patients (mean age: 39+/-11 years) with Behcet's disease who were diagnosed in Asan Medical Center from January 1990 to September 2000. The patients were subgrouped into the mucocutaneous, cardiac, vascular, central nervous system, gastrointestinal and ocular group according to the involved organ. RESULTS: Among 217 patients, nineteen (8.8%) were found to have cardiac involvement: aortic regurgitation was found in ten patients (53%), right ventricular thrombi in six (32%), acute myocardial infarction in two (11%), and myocarditis in one (5%). The incidence of initial oro-genital ulcers at the first clinical presentation was significantly lower in the cardiac Behcet's group than in the other subgroups (52% vs 90%, p=0.001). The event-free survival rate during a mean follow-up duration of 67+/-46 months was also significantly lower in the cardiac Behcet's group (63% vs 14%, p=0.0001). CONCLUSION: Aortic regurgitation and right ventricular thrombus are two common types of cardiac Behcet's disease. Although its incidence is low, cardiac Bechcet's disease has a characteristic pattern for the clinical presentation, and it has a worse prognosis compared with the other subgroups of Behcet's disease that have other solid organ involvement.