Behçet’s disease is a chronic inflamm-atory disease diagnosed by recurrent aphth- ous ulcers, genital ulcers and eye involvement in the form of uveitis.1 Cerebrovascular involvement with arterial aneurysm rupture is an exceedingly rare phenomenon in patients with Behçet’s disease and is only found in 1.6–10% of all vascular lesions in these patients.2,3 Cerebral artery aneurysms, brain parenchymal inflammatory disease, pseudotumour cerebri, intimal hyperplasia of the cerebral artery and vasculitis are the main symptoms of neuro- Behçet’s disease.4 Cardiac involvement leads to inflammatory endocardial disease, coronary artery stenosis, pericarditis, cardiomyopathy and thrombus formation.4 However, the combination of an intra- cardiac thrombus with rupture of a cerebral artery aneurysm is a very rare occurrence.5 Case Report A 35-year-old female known to have Behçet’s disease was referred to the Imam Ali Hospital, Kermanshah, Iran, in July 2014 from the neurosurgery ward for evaluation of postoperative dyspnoea. Seven days previously, she had undergone a cranial surgery for a ruptured cerebral artery aneurysm and the removal of parenchymal haemorrhagic lesions. Upon admission to the neurosurgery ward, an initial diagnosis of a ruptured cerebral artery aneurysm was made based on the parenchymal changes observed on computed tomography [Figure 1]. Cerebral angiography confirm- ed haemorrhagic sequelae in the left cerebral artery branch due to the rupture of the aneurysm [Figure 2]. Seven days after the neurosurgery, the patient complained of vertigo, dysarthria, dyspnoea, fever, 1Preventive Cardiovascular Research Centre Kermanshah and 2Department of Neurology, Kermanshah University of Medical Sciences, Kermanshah, Iran *Corresponding Author e-mail: r.faraji61@gmail.com جلطة يف البطني األمين ومتدد الشريان الدماغي يف مريضة مبرض هبجت فريدون �سابزي، �سامانه مريزايي، ر�سا فرجي abstract: We report a 35-year-old woman referred to the Imam Ali Hospital, Kermanshah, Iran, in July 2014 for evaluation of postoperative dyspnoea after neurosurgery performed seven days previously for a ruptured cerebral artery aneurysm. She was known to have Behçet’s disease with a history of recurrent oral and genital aphthous ulcers and uveitis. At referral, her symptoms included vertigo, dysarthria, palpitations and chest pain. Transthoracic echocardiography (TTE) revealed a large thrombus in her right ventricle outflow tract and open-heart surgery was performed eight days after the previous surgery to remove the clot. The postoperative period was complicated by transient acute renal failure, which resolved spontaneously. The patient was discharged 13 days after the cardiac surgery on warfarin, prednisolone, azathioprine and cyclophosphamide. Cyclophosphamide and azathioprine were discontinued after three months as the symptoms had completely resolved; however, prednisolone was continued due to recurrent uveitis. A 10-month follow-up TTE scan revealed no thrombus recurrence and treatment with warfarin and prednisolone was continued. Keywords: Behçet Disease; Thrombus; Fusiform Aneurysm; Case Report; Iran. امللخ�ص: هذا تقرير حالة لأمراأة تبلغ من العمر 35 عاما مت حتويلها اإىل م�ست�سفى الإمام علي، كرمان�ساه، اإيران، يف يوليو 2014 لتقييم �سيق التنف�س بعد اإجراء جراحة لتمدد �رسياين دماغي منفجر منذ �سبعة اأيام قبل التنومي. كانت املري�سة م�سابة مبر�س بهجت مع تاريخ تلعثم، دوار، من تعاين املري�سة كانت التحويل عند القزحية. والتهاب التنا�سلية والأع�ساء الفم يف املتكررة القالعية للقرح مر�سي وخفقان واأمل يف ال�سدر. ك�سف تخطيط �سدى القلب عرب ال�سدر )TTE( عن وجود جلطة كبرية يف تدفق جماري البطني الأمين. مت اإجراء جراحة قلب مفتوح بعد ثمانية اأيام من اجلراحة ال�سابقة. تعقدت فرتة ما بعد اجلراحة بحدوث الف�سل الكلوي احلاد، الذي �سفي من تلقاء نف�سه. غادرت املري�سة بعد 13 يوما من عملية القلب املفتوح على عقاقري الوارفارين، الربيدنيزولون، الآزوثيوبرين وال�سيكلوفو�سفاميد. مت ايقاف عقاري ال�سيكلوفو�سفاميد والآزوثيوبرين بعد ثالثة اأ�سهر نتيجة لأختفاء كل الأعرا�س متاما. ومع ذلك، ا�ستمر عقار الربيدنيزولون بالوارفارين العالج وا�ستمر اجللطات عودة عن �سهور ع�رسة بعد TTE ال فح�س اعادة تك�سف مل املتكررة. القزحية التهابات ب�سبب والربيدنيزولون. كلمات مفتاحية: مر�س بهجت؛ جلطة؛ متدد الأوعية الدموية املغزيل؛ تقرير حالة؛ اإيران. Right Ventricular Thrombus and Cerebral Artery Aneurysm in a Patient with Behçet’s Disease Feridoun Sabzi,1 Samaneh Mirzaei,2 *Reza Faraji1 case report Sultan Qaboos University Med J, May 2016, Vol. 16, Iss. 2, pp. 250–253, Epub. 15 May 16 Submitted 28 Jun 15 Revisions Req. 13 Aug, 28 Sep & 25 Nov 15; Revisions Recd. 31 Aug, 3 Nov & 13 Dec 15 Accepted 31 Dec 15 doi: 10.18295/squmj.2016.16.02.020 Feridoun Sabzi, Samaneh Mirzaei and Reza Faraji Case Report | e251 with 91% neutrophils; platelet count: 90,000/mm-3 (NR: 150,000–450,000/mm-3); haemoglobin count: 10 g/dL (NR: 13–18 g/dL); C-reactive protein levels: 54 mg/dL (NR: ≤0.8 mg/dL); erythrocyte sedimentation rate: 80 mm/hour (NR: 0–20 mm/hour); blood urea nitrogen levels: 35 mg/dL (NR: 7–25 mg/dL); and creatinine levels: 2.9 mg/dL (NR: 0.6–1.2 mg/dL). The thrombocytopaenia was treated with prednisolone and her platelet levels had normalised when a repeat platelet count was performed three weeks later. Urinalysis revealed proteinuria without haematuria. Anti-nuclear, anti-neutrophil cytoplasmic, anti-double strand and anticardiolipin antibodies were negative, as were rheumatoid factor and lupus anticoagulants. Test results for other thrombophilia factors were normal, including hyperhomocysteinemia, antiphospholipid antibodies, protein S, protein C and antithrombin III levels and factor V Leiden. Transthoracic echocardiography (TTE) revealed a mobile thrombus in the right ventricle outflow tract (RVOT) of the subtricuspid apparatus [Figure 3]; this was believed to have been the cause of the patient’s respiratory symptoms. Eight days after the neurosurgery and one day after admission to the cardiac surgery ward, open cardiac surgery was performed to remove the clot. Intraoperatively, the thrombus was observed to be located in the RVOT with a single site of attachment to the perimembranous septum and septal tricuspid valve [Figure 4]. Surgical exploration of the pulmonary artery and its main branches revealed no other thrombi. A pulmonary thromboembolism was suspected; however, no facil- ities for lung perfusion scans were available and the patient could not be transferred to another centre due to her general condition. Postoperatively, the patient suffered from acute renal dysfunction and nephrologists were consulted. heart palpitations and chest pain and was referred to the cardiac surgery ward. In the ward, her medical history revealed classic features of Behcet’s disease, including four occurrences of mouth and genital ulcers in the previous year and ophthalmologist-confirmed uveitis. A physical examination revealed signs of the former cranial surgery, multiple aphthous mouth and genital ulcers and left haemiparesis. There were no signs of thrombophlebitis or lower limb oedema. Chest radiography was normal. The results of laboratory tests on admission were as follows: white blood cell count: 19,000/mm-3 (normal range [NR]: 4,300–10,800/mm-3) Figure 1: Preoperative computed tomography of a 35-year-old female patient with Behçet’s disease showing a haemorrhagic mass in the right cranial hemisphere (arrow) indicative of a ruptured cerebral artery aneurysm. Figure 2: Postoperative cerebral angiography of a 35-year-old female patient with Behçet’s disease with a ruptured cerebral artery aneurysm in the left middle cerebral artery branch (circle), with aneurysm clip in situ (arrow). Figure 3: Transthoracic echocardiography of a 35-year- old female patient with Behçet’s disease showing a large thrombus (arrows) in the right ventricular outflow tract. LA = left atrium; RA = right atrium; LV = left ventricle; RV = right ventricle. Right Ventricular Thrombus and Cerebral Artery Aneurysm in a Patient with Behçet’s Disease e252 | SQU Medical Journal, May 2016, Volume 16, Issue 2 However, the condition resolved spontaneously. A few days later, the patient was treated with immunosuppressive therapy for symptoms of Behcet’s disease. Symptoms of oral and genital aphthous ulcers and uveitis were relieved using a combination of oral corticosteroids, prednisolone, azathioprine and cyclophosphamide. Nine days after the cardiac surgery, she was prescribed anticoagulation therapy (heparin and warfarin) to prevent further thrombus formation. The patient was discharged on the 13th postoperative day on warfarin, prednisolone, azathioprine and cyclophosphamide. After three months of treatment, the cyclophosphamide and azathioprine were discont- inued due to resolution of the symptoms. At a 10-month follow-up, the patient’s general condition was satisfactory and TTE indicated no thrombus recurrence. However, prednisolone was continued indefinitely due to recurrent eye involvement. Warfarin was also continued due to prolonged bedrest caused by haemiparesis which increases the risk of thromboemboli in the extremities. Discussion This report presents a patient with Behçet’s disease with right ventricle thrombus formation after a previous cerebral artery aneurysm rupture. Cardiac in- volvement in Behçet’s disease is very rare and consists mainly of thrombus formation. While any chamber of the heart may be involved in clot formation, the right atrium, with its unique haemodynamic characteristics, is the most commonly affected chamber.5 All of the layers of the heart are involved in the inflammatory process of Behçet’s disease.6 Endothelial inflammation can cause luminal irregularities and thickening which is revealed as thrombi, Loeffler endocarditis and arterial aneurysm formation. Thrombi of the superior and inferior vena cava (IVC) may cause superior vena cava syndrome and Budd-Chiari syndrome. Systemic lupus erythematosus (SLE) can be associated with cardiac thrombosis and cerebral complications;6 ruling out SLE as a diagnosis was therefore necessary in the current case. Aneurysms of the large and medium arteries— such as the aorta, pulmonary and peripheral arteries including the femoral, popliteal, brachial and subclavian arteries—can form part of the vascular involvement in Behçet’s disease.6 One important medium-sized artery involved in aneurysm formation is the cerebral artery. Although vascular inflammatory disease is observed in only 25% of patients with Behçet’s disease, it is nonetheless the most important predictive factor of Behçet’s disease-related mortality.6 The combination of cerebral and cardiac manifest- ations of Behçet’s disease is a very uncommon phenomenon.7 Intracardiac thrombus formation may be caused by cardiac endothelial inflammation or may arise from an extracardiac thrombus that has broken off from the thromboembolism source in a peripheral vein and dislodged into the cardiac chamber. However, emboli that originate from a cardiac chamber are a relatively uncommon occurrence.7 Mogulkoç et al. detected 13 cases of pulmonary emboli among 25 subjects with Behçet’s disease; in approximately 50% of these cases, thrombosis in the inferior and superior vena cavae and deep lower extremity veins may have been the source of the embolism.5 Most emboli in Behçet’s disease patients begin in the cardiac chambers.8 In patients without Behçet’s disease, deep vein thrombosis in the lower extremities is usually associated with pulmonary emboli, as thrombi formed in the veins in the absence of inflammation can easily break off and travel to the heart and lungs. Contrary to conventional thromboemboli, the combination of a pulmonary thromboembolism with lower extremity phlebitis is a rare event in patients with Behçet’s disease.8 Thrombi formed in inflamed vessels are strongly adherent to the endothelium; therefore, these patients have a low risk of thromboembolism.9,10 Houman et al. observed vascular involvement in 43.3% of 113 Behçet’s disease cases; of those, 89.8% had deep vein thrombosis.11 Involvement of the deep venous system is more common in men than women.10 Luo et al. evaluated the clinical features of patients with Behçet’s disease and cardiac chamber thrombi; they found that intracardiac thrombus formation frequently occurred in young male patients and usually involved the right cardiac chambers.12 In the present case report, the patient was treated with immunosuppressive and antithrombotic drugs following the surgical removal of the cardiac thrombus. Figure 4: Intraoperative photograph of a thrombus in the right ventricular outflow tract of the subtricuspid apparatus (arrow) of a 35-year-old female with Behçet’s disease. Feridoun Sabzi, Samaneh Mirzaei and Reza Faraji Case Report | e253 metabolomic evaluation of Behcet’s disease with arthritis and seronegative arthritis using synovial fluid. PLoS One 2015; 10:e0135856. doi: 10.1371/journal.pone.0135856. 4. Erkan F, Gül A, Tasali E. Pulmonary manifestations of Behçet’s disease. Thorax 2001; 56:572–8. doi: 10.1136/thorax.56.7.572. 5. Mogulkoç N, Burgess MI, Bishop PW. Intracardiac thrombus in Behçet’s disease: A systematic review. Chest 2000; 118:479–87. doi: 10.1378/chest.118.2.479. 6. Aşker S, Aşker M, Gürsu O, Mercan R, Timuçin OB. A Behcet’s disease patient with right ventricular thrombus, pulmonary artery aneurysms, and deep vein thrombosis complicating recurrent pulmonary thromboembolism. Case Rep Pulmonol 2013; 2013:492321. doi: 10.1155/2013/492321. 7. Franco-Macías E, Roldán-Lora F, Martínez-Agregado P, Cerdá- Fuertes N, Moniche F. Neuro-Behçet: Pons involvement with longitudinal extension to midbrain and hypertrophic olivary degeneration. Case Rep Neurol 2015; 7:148–51. doi: 10.1159/000435804. 8. Düzgün N, Küçükşahin O, Atasoy KÇ, Togay Işıkay C, Gerede DM, Erden A, et al. Behçet’s disease and intracardiac thrombosis: A report of three cases. Case Rep Rheumatol 2013; 2013:637015. doi: 10.1155/2013/637015. 9. Bouali E, Kaabachi W, Hamzaoui A, Hamzaoui K. Inter- leukin-37 expression is decreased in Behçet’s disease and is associated with inflammation. Immunol Lett 2015; 167:87–94. doi: 10.1016/j.imlet.2015.08.001. 10. Joo HJ, Hong SJ, Yu CW. Transcatheter aortic valve-in-valve implantation for severe bioprosthetic stenosis after Bentall operation using a homograft in a patient with Behçet’s disease. J Heart Valve Dis 2015; 24:217–19. 11. Houman MH, Ben Ghorbel I, Khiari Ben Salah I, Lamloum M, Ben Ahmed M, Miled M. Deep vein thrombosis in Behçet’s disease. Clin Exp Rheumatol 2001; 19:S48–50. 12. Luo L, Ge Y, Liu ZY, Liu YT, Li TS. [A report of eight cases of Behcet’s disease with intracardiac thrombus and literatures review]. Zhonghua Nei Ke Za Zhi 2011; 50:914–17. Cyclophosphamide, azathioprine and prednisolone were prescribed. Prior to starting treatment with cyclophosphamide and azathioprine, routine baseline assessments were performed, including a complete blood cell count and liver and renal function tests; regular monitoring continued throughout treatment. To prevent urinary toxicity such as haemorrhagic cystitis, patients need to be adequately hydrated and pass urine frequently. As such, patients should be instructed to increase their fluid intake 24 hours before, during and at least 24 hours after receiving cyclophosphamide.12 Conclusion In the present case, a large intracardiac thrombus was detected in a patient with Behçet’s disease who had previously undergone neurosurgery for a ruptured cerebral artery aneurysm. The patient underwent open- heart surgery for the removal of the thrombus before undergoing immune suppression and antithombotic therapy. At a 10-month follow-up, she was healthy with no evidence of further thrombus formation. References 1. Mahfoudhi M, Turki S. [Giant aneurysm of the pulmonary artery revealing Behçet syndrome]. Pan Afr Med J 2015; 20:411. doi: 10.11604/pamj.2015.20.411.5941. 2. Mahfoudhi M, Goucha R. [Behçet syndrome associated with IgA nephropathy]. Pan Afr Med J 2015; 20:410. doi: 10.11604/ pamj.2015.20.410.5956. 3. Ahn JK, Kim S, Kim J, Hwang J, Kim KH, Cha HS. 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