Association of endomyocardial fibrosis and minor myocarditis sequelae with intracardiac thrombus and Ebstein like valvulopathy in a patient with Behçet disease: a case report

Cardiac complications occur in 1–6% of cases of Behçet disease (BD) with intracardiac thrombus being the most frequent complication. Endomyocardial fibrosis, less common and occasionally associated with intracardiac thrombus, is reported in <20 case reports of BD, among which, three cases are described to mimic Ebstein disease based on echocardiography. We present the first case in the literature of a 34-year-old man with BD diagnosed with multiple cardiovascular complications, highlighting the challenging diagnosis and treatment of this pathology, especially regarding anticoagulation therapy.

A 34-year-old man, diagnosed with BD, presented to the Emergency Room with haemoptysis. Computed tomography study of the thorax diagnosed pulmonary arterial aneurysm with multiple arterial thrombi, associated with multiple intracardiac thrombi in the right ventricle and atrium. The echocardiography confirmed the presence of voluminous thrombi in the right ventricle and atrium and showed hypertrabeculation of the right ventricle and a high insertion of the posterior leaflet of the tricuspid valve inducing a moderate tricuspid insufficiency compatible with an Ebstein disease. The cardiac MRI later revealed right ventricular fibrosis consistent with endomyocardial fibrosis and sequelae of myocarditis, also described as BD rare cardiac manifestations. The patient had a favourable outcome under anticoagulant treatment and immunosuppressive drugs.

The association of multiple cardiovascular complications can occur in a single patient with BD. The endomyocardial fibrosis in the right heart chambers acting as a substrate for thrombus formation and subsequent pulmonary embolism; fibrosis extending to the tricuspid valve inducing an Ebstein-like morphology.