Behçet's Disease and Intracardiac Thrombosis: A Report of Three Cases

Abstract

We present three patients with Behçet's disease associated with intracardiac thrombus and pulmonary vascular involvement. One of these patients had also Budd-Chiari syndrome. All patients were treated with corticosteroid plus monthly intravenous cyclophosphamide as first line treatment and with no recurrences. Immunosuppressive therapy was successful in the treatment of intracardiac thrombus and also in the regression of pulmonary vascular thromboses in these patients. Intracardiac thrombus in Behçet's disease is rarely seen. Behçet's disease should be remembered in the differential diagnosis of the patients with intracardiac mass, especially in patients from the Mediterranean and Middle East populations.

Figure 1

(a) Transthoracic echocardiographic apical four-chamber view shows a thrombus in the apex of the left ventricle. (b) Four-chamber-view MR image obtained with fast imaging steady-state free precession sequence shows pedunculated thrombus (arrow) attached to left ventricular apex with a thin stalk (cine images showed mobility of the thrombus throughout the cardiac cycle). (c) Pulmonary CT angiography shows acute embolus as a filling defect in the left lower lobe artery (arrow). Note the associated left pleural effusion (PE). (d) Lung window setting shows bilateral lower lobe infarcts, more prominent in the left lung.

Figure 2

(a) Transthoracic echocardiography in the parasternal short-axis view shows a thrombus (arrow) in the right ventricle attached to the interventricular septum. (b) Contrast-enhanced pulmonary CT angiography shows acute embolus filling and mildly enlarging the right middle lobe artery (arrow).

Figure 3

(a) Transthoracic echocardiography showed a mobile mass filling the half of the right atrium (4.5 × 3 cm in size) (arrow) extending the right ventricule (1.5 × 1.6 cm in size) and into the vena cava. (b) Contrast-enhanced CT angiographic image shows a large filling defect in the right atrium extending into the right ventricle (asterisks). Segmental arteries in both lower lobes are of small calibre due to chronic thromboembolism (arrows). Pulmonary hypertension resulted in straightening of the interventricular septum. (c) Both lower lobe arteries contain central filling defects (arrows) representing superimposed acute pulmonary embolism.