Elsevier

Canadian Journal of Cardiology

Volume 30, Issue 10, October 2014, Pages 1250.e1-1250.e3
Canadian Journal of Cardiology

Case Report
Recurrent Right Atrial Thrombosis Due to Behçet Disease

https://doi.org/10.1016/j.cjca.2014.05.011Get rights and content

Abstract

Behçet disease is a rare condition sometimes associated with chronic cardiac inflammation followed by myocardial dysfunction and vascular inflammation. We report a case of recurrent right atrial thrombus due to Behçet disease despite continued anticoagulation therapy. The thrombus disappeared after the initiation of immunosuppressive therapy. To avoid a progression to thrombus or cardiac dysfunction in this recurrent case, the early identification of cardiac involvement of Behçet disease using echocardiography and/or cardiac magnetic resonance imaging might be important. Combined immunosuppressive therapy with prednisone and cyclophosphamide might be needed to treat recurrent thrombosis due to Behçet disease.

Résumé

La maladie de Behçet est une affection rare quelquefois associée à une inflammation chronique du cœur qui est suivie d’une dysfonction myocardique et d’une inflammation vasculaire. Nous présentons un cas de thrombus auriculaire droit récurrent dû à la maladie de Behçet en dépit d’une anticoagulothérapie continue. Le thrombus est disparu après l’introduction de la thérapie immunosuppressive. Pour éviter une progression vers un thrombus ou une dysfonction cardiaque de ce cas récurrent, la détection précoce de la participation cardiaque de la maladie de Behçet à l’aide de l’échocardiographie ou de l’imagerie cardiaque par résonance magnétique, ou les deux, serait importante. La thérapie immunosuppressive combinée à la prednisone et à la cyclophosphamide serait nécessaire au traitement de la thrombose récurrente due à la maladie de Behçet.

Section snippets

Case Report

In Year 1, a 45-year-old man presented with symptoms of orogenital ulcers, erythema nodosa, and bilateral painful swelling in his legs diagnosed as deep vein thrombosis (DVT). Anticoagulation therapy was initiated and echocardiography showed no cardiac mass. In Year 2, he was readmitted with high-grade fever and recurrence of DVT, and echocardiography revealed a large mass (70 × 60 mm) in the right atrium. Protein C, protein S, and thrombomodulin levels were normal and antinuclear and

Discussion

In the present case, an atrial thrombus recurred because of reactivation of Behçet disease, despite continuous administration of anticoagulant medication (warfarin, international normalized ratio target maintained at 2.0-3.0) and prednisone. Recurrence of disease in this case was supported by the presence of an intracardiac mass, myocardial dysfunction, DVT, and gastrointestinal ulcers. Behçet disease has been reported to cause myocardial inflammation that results in myocardial dysfunction.1, 2

Funding Sources

This report was supported by Health Labor Sciences Research Grant, Japan.

Disclosures

The authors have no conflicts of interest to disclose.

References (5)

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