Abstract
BACKGROUND: Behçet's disease BD is a rare multisystemic disease, with rare cardiac involvement. This case illustrates a rare cardiac involvement as a giant intracavitary thrombus which was the revealing form of Behçet disease. CASE PRESENTATION: An 15-year-old male admitted to the emergency department for progressive dyspnoea, hemoptysis for which an echocardiogram displayed a large echogenic mass in the right ventricle and angio CT revealed associated bilateral pulmonary embolism. The patient was then proposed for surgery for removal and pathological study the later confirmed its fibrin thrombotic nature. Behçet disease was suspected based on past history of recurrent oral aphthosis and confirmed with a positive pathergy test. Further management by anticoagulants, immunosuppressants and corticosteroids seemed effective to avoid relapse. CONCLUSIONS: Cardiac involvement during BD can be life-threatening as it is not always diagnosed in timely manner. However, intracardiac thrombus is uncommon with only few case reports. Echocardiography is the key tool for the diagnosis of intracardiac thrombus.