Intracardiac Thrombus in Behçet's Disease.

Behçet's disease (BD) is a chronic multisystem disease that presents with recurrent oral and genital ulceration and recurrent uveitis. The patients are often diagnosed in the range of 20-30 years of age and BD are more common in men.[1] BD has a worldwide distribution, but it is mainly observed in Mediterranean areas and Japan.[2] Involvement of skin, joints, nervous, respiratory, gastrointestinal, and cardiovascular systems is also recognised.[34] Although the vascular lesions are frequently observed in this disease, the cardiac involvement is rare and is associated with the poor prognosis.[5].

INTRODUCTION

We report a case of a man with Behçet's disease (BD) with unusual manifestations and rare complications, discovered by an intracardiac thrombus associated with a pulmonary embolism.

CASE REPORT

A 31-year-old man who had previously received a diagnosis of BD was admitted to our hospital with a 3 weeks history of dyspnea, cough, orthopnea and haemoptysis. A diagnosis of Behçet syndrome had been established 3 years earlier, on the basis of recurrent genital and oral ulcers with a positive pathergy test and HLA-B5 genotype. He had been treated with corticosteroids and colchicine.

On physical examination, the patient had breathlessness. His temperature of body was 37.8°, blood pressure was 100/75 mmHg, respiration rate was 23/min, and pulse rate was 105/min, and regular. There was no finding on cardiac and lung examination. The electrocardiogram showed a sinus tachycardia with a right bundle branch block. The X-ray chest showed mild hilar enlargement. Venous Doppler ultrasound was performed and no venous thrombosis was detected. The laboratory tests revealed an inflammatory syndrome, and positive HLA-B51. C-reactive protein was 27 mg/L; erythrocyte sedimentation rate was 63 min. Liver and kidney function tests were normal. The patient was negative for factor V Leiden, anti-endothelial cell antibodies, anti-cardiolipin antibodies, prothrombin mutation, von Willebrand factor, and lupus anticoagulant. The protein C and S levels were within the normal limits.

The echocardiography showed a normal left ventricle ejection fraction, no vegetations were found on the heart valves. A right ventricle enlargement and pulmonary arterial hypertension (47 mmHG) were detected. We saw a 2,1 cm × 2 cm mass in the middle of the right ventricle [Figure 1]. Pulmonary embolism was considered with these findings. Then, the diagnosis of pulmonary embolism was confirmed with the thoracic spiral computed tomographic, and thrombus was shown in the right pulmonary artery [Figure 2]. The patient was treated with low molecular weighted heparin, oral cyclophosphamide (100 mg/day), and prednisone (1 mg/kg/day). The glucocorticoid dose was gradually reduced. The patient's symptoms improved dramatically. The thrombus in the right ventricle decreased considerably in size before discharge. In addition, warfarin was started, and administered to maintain the international normalized ratio at 2.5 or above. Three months later, the right ventricular thrombus, and pulmonary hypertension had disappeared on transthoracic echocardiography [Figure 3]. The patient was still being monitored, without recurrence, 24 months later.

Figure 1

The echocardiography showed a normal left ventricle ejection fraction, no vegetations were found on the heart valves and a mild right ventricle enlargement with a 21 cm × 2 cm mass in the middle of the right ventricle

Figure 2

Thoracic spiral computed tomographic was performed, and thrombus was shown in the right pulmonary artery and pulmonary embolism was confirmed

Figure 3

Three months later, the right ventricular thrombus and pulmonary hypertension had disappeared on transthoracic echocardiography

DISCUSSION

The prevalence of cardiovascular involvement in BD between changes 7% and 29% according to the different ethnic group.[67] And the prevalence of vascular involvement varies from 7.7% to 43%, according to the ethnicity of the population.[7] In one of the largest case series published so far, 52 (6%) patients from a cohort of 807 patients with BD had some form of cardiac involvement including myocardial infarction, endocarditis, pericarditis, endomyocardial fibrosis, and aneurysms of the coronary arteries of sinus of Valsalva.[7] However, intracardiac thrombosis is a rare finding with <100 cases reported in the medical literature.[8] Besides, intracardiac thrombi were also associated with thrombosis of the vena cava and pulmonary embolism (28% and 60% of cases, respectively).[8910] The clinical manifestations of the disease start with fever (50.9%), hemoptysis (55.4%), and dyspnea (36.2%). It can be associated with a venous thrombosis of the superior and/or inferior vena cava (45.3%), pulmonary embolism (41.5%), and aneurysms of the pulmonary arteries (38.3%).[9] It usually involves the right side of the heart (right chambers in 78% of cases: The right atrial chamber)[11] and the right ventricle,[12131415] more often than the left ventricle.[1617] The involvement of the left heart has rarely been reported or discussed.[161718]

Transesophageal and transthoracic echocardiography are generally enough to enable a diagnosis of cardiac thrombosis. The mass is usually heterogeneous and echogenic, involving the ventricles rather than the atriums[18] and the right side more often than the left.[19] In our patient, the thrombus was found in the right ventricle.

There is no consensus regarding the treatment of cardiac thrombosis associated with BD.[12] The aim is to clean the cardiac thrombus and to prevent the recurrence of this complication. Anticoagulants alone do not seem to be an effective treatment; systemic glucocorticoid and immunosuppressive agents are highly recommended before using the anticoagulants.[20] Surgery might become necessary in case of cardiac thrombosis that is massive, and recurrent after medical treatment, or is associated with the cardiac congestion.[1]

CONCLUSION

Early echocardiography is advisable for the detection of cardiac involvement, and the medical treatment should be the first choice of therapy. It is critical to keep in mind that the Behçet's syndrome in patients with intracardiac thrombus, in order to institute the appropriate medical treatment as soon as possible. The combination of methylprednisolone, cyclophosphamide, and warfarin seems to be a good option to treat such a complication.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.