Abstract Background and Aims Behçet syndrome (BS) is a complex multisystemic vasculitis of unknown exact etiology. Cardiac involvement (CI) is rarely described, yet associated with high morbidity and mortality rates. Our work aims to depict the profile and outcomes of BS patients presenting with cardiac lesions. Methods This descriptive and monocentric study was retrospectively carried out between 2000 and 2022, among 531 patients admitted to our department for BS. Results 32 cases of CI were enrolled (6%). The sex ratio (M/F) was 3:1 and the mean age was 30 ± 7 years (21-48). Cardiovascular risk factors comprised smoking (21%), high blood pressure (15%), and diabetes mellitus (11%). CI was mostly asymptomatic (71%), discovered during systematic screening. Otherwise, patients presented with chest pain (21%), dyspnea (19%), dry cough (18%), and hemoptysis (17%). Inflammatory parameters were elevated in 71% of the cases, with a median ESR of 46 mm/h and CRP of 55 mg/l. Hemostasis parameters were within the normal ranges for all patients. The lesions visualized were right intracardiac thrombosis (79%), pericarditis (34%), coronary thrombosis (12.5%), myocarditis (6%), and endomyocardial fibrosis (3%). Concomitant vascular involvement was frequent (72%), especially thrombotic lesions in the inferior vena cava (23%), pulmonary arteries (21%), lower extremities (15%), cerebral veins (18%), and superior vena cava (9.5%). Arterial aneurysms were also common, mostly located in the pulmonary arteries (25%). Noncardiovascular involvements were mucocutaneous (78%), articular (28%), ocular (43%), neurological (18%), and digestive (3.6%). Patients were treated with colchicine (96%), glucocorticoids (96%), curative anticoagulation (54%), cyclophosphamide (57%), azathioprine (46%), and TNF-a inhibitor (3%). No patient underwent an endovascular procedure. Surgery was performed in 6.3% of the cases. One relapse of intracardiac thrombosis was recorded. Death was documented in five cases. Conclusions CI is a serious manifestation of BS. Although rare, it is also fairly underdiagnosed due to its nonspecific features and discrete evolution. Hence, better knowledge of the disease is crucial for early diagnosis and prompt initiation of immunosuppressive therapy.
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