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ISSN: 2327-5146
General Medicine: Open Access
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Cardiac and Cerebral thrombosis in Behcet’s Syndrome

Freidoun Sabzi, Fahime ghasemi and Reza Faraji*
Department of Cardiovascular Surgery, Imam Ali Heart Center, Kermanshah University of Medical Sciences, Kermanshah, Iran
Corresponding Author : Sabzi F
Department of Cardiovascular Surgery
Imam Ali Heart Center
Kermanshah University of Medical Sciences
Kermanshah, Iran
Tel: +98 831 8360295;
Fax: +98 831 9360043;
E-mail: feridoun [email protected]
ReceivedMay 27, 2014; Accepted July 30, 2014; Published August 20, 2014
Citation: Sabzi F, Ghasemi F, Faraji R (2014) Cardiac and Cerebral thrombosis in Behcet’s Syndrome. Gen Med (Los Angel) 2:145. doi: 10.4172/2327-5146.1000145
Copyright: 2014 Sabzi F, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
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Abstract

A 26-year-old man without history of Behcet’s syndrome was admitted to our department with a history of headache, fever, dyspnea, and weight loss. Transthoracic echocardiography and brain magnetic resonance imaging revealed a right atrial thrombus and sagital sinus thrombosis respectively. Surgery revealed the mass to be a thrombus. After 1 month of treatment with warfarin, he had experienced right atrial clot again. The von Willebrand factor antigen level was found to be elevated in blood examination. The large right atrial clot resected by second open heart surgery and medical treatment continued with cyclophosphamide, and corticosteroid, and thrombus did not reoccur in 6-month follow-up. To the best of our knowledge this is the first case of cerebral vein thrombosis associated with right atrial clot formation.

Keywords
Behcet’s syndrome; Cardiac; Cerebral thrombosis; Sagittal sinus; Heart surgery
Introduction
Behcet’s disease is an autoimmune and inflammatory disease of unknown etiology. It is a generalized disorder affects mainly young adults in Mediterranean, Middle Eastern, countries as Iran. It is defined by Mogulk as recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions [1,2]. As per Ames and colleagues Behcet’s disease as a multisystem disorder may affect any organ in different combinations. Involvement of the heart is rare but combination of right atrium and sagital sinus thrombosis are exceptional. Careful medical literature review revealed no such case [3]. Other cardiac pathology that described by Ozkan et al. [4] and ourkan et al., Goktekin et al. includes, aortic mitral valve prolepses, aortic root involvement, aortic valve regurgitation, coronary artery aneurysm, true myocardial aneurysm pericarditis, myocarditis, endocarditis, endomyocardial fibrosis, conduction system disturbances such as left or right bundle branch block or hemi block or complete heart block, coronary arteritis, acute myocardial infarction, and dilated cardiomyopathy [4,5]. Islim et al. [7] also found left ventricular diastolic dysfunction in echocardiography measurements.
Case Report
We report a patient who was admitted to the emergency unit of our cardiac surgery center. Patient did not have a history of the oral aphtous, or genitalia or skin ulcer. However, patient was found to have headache, fever, weight loss, and fatigue. Echocardiographic examination revealed a non-mobile mass attached to right atrium near coronary sinus ostium that was initially thought to be an infected clot in unusual location, and the patient’s condition was consulted with infectious disease specialists and neurologist. (Figure 1) Patient underwent surgery which revealed the mass to be a thrombus.
Brain MRI revealed sagital sinus thrombosis and patency of cerebral artery (Figure 2-4). After 4 weeks of anticoagulation with oral warfarin (7.5 mg) daily and the target INR was 3.0 to 3.5 and antibiotics therapy, patient was readmitted to our center with the oral, genitalia and skin ulcers. Additionally, a right atrial thrombus was found again on echocardiographic examination. A careful reevaluation revealed pustular lesions on his skin and laboratory exam revealed very high level of von willibrand factor. The thrombophilia study did not show any abnormalities, and anti-nuclear, anti-DNA, anti-ENA, and anti-neutrophil cytoplasm antibody tests were all negative. Given these findings, the patient was diagnosed with Behçet’s disease (BD). An immediate transthoracic echocardiogram (TTE) revealed normal left ventricular function (EF, 55%), right ventricular normal pressure, normal RV, and interventricular septum function with absent left ventricular or left atrial clot. Electro cardiogram (ECG) revealed normal sinus rhythm. The large right atrial clot was resected by second open heart surgery and medical treatment was continued with immunosuppressive and anticoagulation therapy. Prednisone at 1 mg/kg/day was added to the treatment, yielding immediate improvement in clinical and laboratory parameters, and the acneiform lesions cleared up. Six months later, the patient was asymptomatic, and was being treated with anticoagulants, azathioprine, colchicine, and prednisone (5 mg/day). After 6 month follow-up, echocardiographic examination showed complete disappearance of thrombus.
Our patient fulfilled criteria of behcet disease by: recurrent oral apthous i.e. three times in six month follow-up. In addition, he also meets two of the following: 1-recurrent genital ulcerations 2-skin lesions (acneiform nodules)
Discussion
Kiriz et al. [8] studied the pathological homeostasis and “prothrombotic state” in Behcet’s disease. The found elevated von Willebrand factor antigen levels to be the most important causative factor for clot formation in vascular system. Gul et al. [9] found that coagulation factor V gene mutation increases the risk of venous thrombosis in Behcet’s disease. In addition, fibrinolysis anomaly due to endothelial cell damage causes deposition of antigen-antibody and immune complex in sub endothelial area which leads to thrombosis [9]. Hammami and colleagues found that the risk of homozygosis of factor V Leiden mutation and the prothrombin gene is high in Behcet’s syndrome which increases the risk of thrombosis by a factor of 6 and 3, respectively [10]. Koc et al. found that the range of cardiac involvement in Behcet syndrome varies from 1% to 5 %. The precedence of cardiac involvement along with conventional and systemic signs and symptom such as uveitis, oral and genitalia ulcer makes diagnosis of Behcet’s disease difficult [11]. Yue described types of cardiac mass in Behcet’s disease. These were: intra cardiac thrombosis, endomyocardial fibrosis, endocardial fibroelastosis, and inflammatory mass and cystic changes [12]. Erer reported the case of a 32-yearold man with newly diagnosed Behcet’s disease (BD) with cardiac, central nervous system and vascular involvement. Similar to our study, Erer et al. [13] found that the neurologic complications related to paranchymal involvement in Behcet disease and not related to venous system thrombosis. Chang et al. reported a 54-year-old man with an established diagnosis of Behcet’s disease who presented with multiple cardiovascular complications such as extensive venous occlusions, right ventricular thrombosis, multiple pulmonary thromboembolisms, and a pulmonary artery aneurysm [14]. Marc et al. reported a case of 60 year old male patient with Behcet’s disease who presented with dyspnea and haemoptysis. CT angiography and echocardiography was performed allowing the identification of right heart thrombus associated with pulmonary artery aneurysm [15]. The outcome was favorable with medical management.
Conclusion
Combined cardiac and paranchymal brain involvement in BD patients has been previously reported in single case report but association of right atrial clot with cerebral veins thrombosis has not been reported so far. Diagnosis of BD is extremely difficult due to lack of any laboratory test. However in some patients, as in our case, serum level of von Wilibrand factor could aid in diagnosis of BD. Intracardiac thrombus formation combined with sagital veins thrombosis, as seen in our patient, is exceptional even among cardiovascular cases of Behcet’s disease
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