Cardiovascular manifestations in juvenile-onset Behçet's disease: unusual mode of revelation

Abstract

Background: Behçet’s disease is a systemic vasculitis with unusual thrombosis, especially in children. Intracardiac localization is rare. The site of choice is the right ventricle. It may be isolated, but must necessarily seek other vascular attacks, in particular an aneurysmal pathology of pulmonary arteries. Its clinical expression is not specific. Case Presentation: An 8-years-old boy was hospitalized for an isolated prolonged fever with marked inflammatory state. No infectious symptoms were identified. Electrocardiogram showed a right branch block and negative T waves in V1-V3 leads. Transthoracic echocardiography revealed multiple right ventricular masses, associated with minimal pericardial effusion. These masses have various sizes and are lining right side of interventricular septum and pulmonary infundibulum without right ventricular outflow tract obstruction. Thoracic computerized tomography scan and cardiac magnetic resonance imaging confirmed that cardiac masses were multiples thrombi filling right ventricle and pulmonary artery. Thrombophilia panel assessment and eye fundus examination were normal. The patient was not a carrier of the HLA B51 gene. Juvenile Behcet’s disease was the final diagnostic. Treated by anticoagulant and corticotherapy, the clinical and ultrasound course of the patient was favorable. A 3-year follow up didn’t show a recurrence of these thrombi. Conclusion: Regression of thrombus under anticoagulant, of fever and inflammatory syndrome under corticosteroid therapy, is a good retrospective diagnostic criterion.