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 Table of Contents  
Year : 2014  |  Volume : 26  |  Issue : 2  |  Page : 88-90

Left main coronary artery thrombosis revealing angio-Behçet syndrome

Department of Cardiological Investigations and Resuscitation, La Rabta University Hospital, Tunis, Tunisia

Date of Submission12-Apr-2014
Date of Acceptance16-Apr-2014
Date of Web Publication28-Aug-2014

Correspondence Address:
Jihen Ayari
Department of Cardiological Investigations and Resuscitation, La Rabta University Hospital, 1007, Jebbari-Tunis, Tunis
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1110-7782.139578

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Although acute myocardial infarction commonly results from coronary atherothrombosis, there are several other etiologies that should be taken initially into account, especially in young adults without significant atherosclerotic risk factors. Thrombophilia and coronary arteritis are, in this context, examples of etiologies that should be looked after. Through this article, we present a case of Behçet's disease with arterial involvement diagnosed after myocardial infarction resulting from thrombosis of the left main coronary artery in a 38-year-old young man without any particular past medical history.

Keywords: Behçet′s disease, left main coronary artery, ST-elevation myocardial infarction, thrombolytic therapy, thrombosis

How to cite this article:
Ayari J, Mourali MS, Farhati A, Mechmeche R. Left main coronary artery thrombosis revealing angio-Behçet syndrome. Egypt J Intern Med 2014;26:88-90

How to cite this URL:
Ayari J, Mourali MS, Farhati A, Mechmeche R. Left main coronary artery thrombosis revealing angio-Behçet syndrome. Egypt J Intern Med [serial online] 2014 [cited 2020 May 31];26:88-90. Available from: http://www.esim.eg.net/text.asp?2014/26/2/88/139578

  Introduction Top

Behçet's disease (BD) represents a frequent systemic vasculitis in Japan and Mediterranean basin. It commonly associates buccal and genital ulcers with cutaneous, ocular, neurological, vascular, and gastrointestinal lesions. Although vasculitis represents the anatomic substratum of BD, its presence is not necessary to make diagnosis. In such disease, the incidence of vasculitis varied from 7 to 29% [1], with marked predominance of venous lesions [2]. Coronary involvement was rarely reported [3-5]. Literature data in this regard are scarce; hence, real incidence of coronary occlusion in BD remains unknown. Our article illustrates the management of angio-Behçet, diagnosis of which was made following ST-elevation myocardial infarction (STEMI) secondary to left main thrombosis.

  Case report Top

We report the case of a male patient aged 38 years, without any particular past medical history and having active smoking (10 pack-years) as the only atherosclerosis risk factor. This patient presented in August 2011 with acute and constrictive chest pain evolving since 3 h and highly evocative of infarction scene. Physical examination noted initially stable hemodynamic status without signs of heart failure. On ECG [Figure 1], ST-segment elevation of about 5 mm in anterior leads and mirror images in inferior leads were noted. This presentation was consistent with the diagnosis of a noncomplicated anterior STEMI seen at the third hour. Given the fact that primary angioplasty was not available at that hour, we immediately opted for pharmacologic revascularization by streptokinase. After fibrinolysis, clinical and electric success criteria were obtained. The course was marked by setting of cardiogenic shock and nonsustained ventricular tachycardia. On transthoracic echocardiography performed in extreme urgency, there were not any mechanical complications. Transthoracic echocardiography showed, however, extended akinesis of the anterior and lateral walls and of the apex without intraventricular thrombus. Left ventricle ejection fraction was estimated at 25%. At the fourth hour and 30th minute, the patient underwent emergency coronary angiography and intra-aortic counterpulsion balloon. The former showed a fresh thrombus floating in the distal part of the left main with TIMI3 flow [Figure 2] and angiographically healthy coronary arteries. The patient was put on antiglycoprotein IIb-IIIa (anti-GpIIb-IIIa) (tirofiban) during 48 h. At the fifth hour following fibrinolysis, peak creatine phosphokinase and troponine Ic amounted, respectively, for 8353 IU/l and 322 ng/l. C-reactive protein and erythrocyte sedimentation rate amounted, respectively, for 82 mg/l (first day of STEMI) and 94 mm/h (second day postmyocardial infarction). Dobutamine and intra-aortic counterpulsion balloon were withdrawn on the fourth day postmyocardial infarction.
Figure 1:

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Figure 2:

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On the seventh day post-STEMI, control coronary angiography showed angiographically healthy and patent coronary arteries [Figure 3]. Etiological investigation did not reveal other atherothrombosis risk factors such as diabetes mellitus or dyslipidemia. Similarly, screening of hyperhomocysteinemia and of anticardiolipin antibodies, anti-β2 microglobulin-1 antibodies, and protein S, C, and antithrombin deficiencies was negative. Careful questioning and physical examination revealed bipolar ulcers (buccal with a scrotal scar consistent with a sequel of scrotal ulcer), which were highly evocative of BD; hence, it was associated with a positive pathergic test and inflammatory arthralgias. Thoracoabdominopelvic multislice computed tomography scan did not draw evidence of other arterial or venous involvement such as aneurysms or occlusions. There were no signs of uveitis on eye fundus. Given this body of evidence, the diagnosis of angio-Behçet was retained. As a consequence, the patient received, in addition to anti-ischemic medications, colchicine 1 mg/day and boli of solumedrol for 3 consecutive days. After that, he was put on oral corticotherapy (prednisone) with monthly boli of cyclophosphamide. The patient was, moreover, under double antiplatelet therapy (acetyl salicylate and clopidogrel), bisoprolol, furosemide, enalapril, and atorvastatin. Clinical course was good; the patient did not develop signs of left heart failure, and repeated echocardiographic controls showed improved and stable left ventricle ejection fraction at 40%.
Figure 3:

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  Discussion Top

The incidence of vascular involvement in BD varies from 7 to 29% [1]. Cardiac involvement is present in 0.14% of patients [1]. Intraventricular thrombosis, regurgitant valvular heart diseases, coronary arteries aneurysms, and myocardial infarction are some of the reported manifestations of cardiac involvement in such disease [6]. Sporadic cases of granulomatous endocarditis, myocarditis, pericarditis, aortic aneurysms, and conductive tissue disorders were also reported during BD [5].

In such a disease, arterial involvement is less frequent than venous lesions. However, it is markedly more dreadful, as it was correlated to an important morbimortality. There are two types of arterial manifestations: occlusive or more commonly aneurismal lesions, which were noted in 1.5-2.2% of patients [7]. Occlusive and stenotic lesions involve not only great vessels, but also vasa vasorum. Frequently involved arteries in decreasing order are pulmonary artery, femoral, popliteal, subclavian, and carotid arteries. Coronary arteries, particularly the left main coronary artery, are rarely involved. Three forms of coronary arteries involvement during BD were reported: stenosis, thrombosis, and pseudoaneurysms. There are two mechanisms to explain thrombosis during angio-Behçet: vasculitis and hypercoagulable state. BD's vasculitis involves usually all layers of a vessel and is characterized by the presence of lymphocytic infiltrate during the acute phase. At an advanced stage, an important fibrotic and scarring reaction develops [8],[9]. Hypercoagulable state observed during BD is thought to be due to inhibition of fibrinolysis on one hand and due to increased platelet aggregation on the other hand. The latter had been explained by endothelial dysfunction leading to increased production of von Willebrand factor and decreased levels of prostacyclin (PGI2) [9,10]. Increased production of fibrinogen and factor VIII could also represent other etiological factors explaining hypercoagulable state [11]. In our case, both hypercoagulable state and vasculitis seemed to be involved in coronary occlusion.

In a young patient with almost no significant risk factors of atherosclerosis, etiologies of acute coronary syndrome are, amongst others, coronary embolism, spasm or dissection, and also anomalous coronary arteries. Attention will be focused toward coronary arteritis when there is a particular context of systemic involvement. In our patient, thrombophilia was highly suspected, given angiographically healthy coronary arteries associated with important thrombotic burden in the left main. As biochemical assays performed to search for hyperhomocysteinemia and protein C, S, and antithrombin deficiencies were normal, the discovery of valuable signs evocative of BD allowed us to retain the diagnosis of angio-Behçet according to the reported criteria by the international study group for BD [12].

As cases are scarce, optimal therapeutic alternative in case of coronary thrombosis during BD remains unknown. Some authors suggested, however, that primary angioplasty was the most recognized revascularization option during STEMI secondary to BD's coronary thrombosis [13],[14],[15]. Nevertheless, long-term results after successful angioplasty and stent implantation remain unknown [16]. According to Ando et al. [17], coronary bypass grafting was not considered the best therapeutic option, as it was associated with postoperative coronary pseudoaneurysms. In our patient, in whom BD was unknown at admission, pharmacologic revascularization was undertaken at the acute phase of STEMI and was effective as demonstrated by the clinical and electric criteria on one hand and by angiographic findings (TIMI3 flow) on the other hand. Given the marked thrombotic burden in the left main, anti-GpIIb-IIIa (tirofiban) was given. Similarly, other successful cases of fibrinolysis were reported [18] in addition to efficiency of anti-GpIIb-IIIa [19], which was, in preference, indicated in case of fresh thrombus floating in coronary arteries without delayed flow or need for emergent revascularization. Moreover, according to the case reported by Ergelen et al. [20] illustrating left main thrombosis with stable hemodynamic status in a patient suffering from BD, successful fibrinolysis followed by the use of anti-GpIIb-IIIa had proved effective as demonstrated by further coronary angiography showing patent coronary arteries. Because of the arterial involvement, BD was considered severe in our patient justifying initial intravenous corticotherapy followed by oral corticoids and cyclophosphamide. Nevertheless, although steroidal anti-inflammatory drugs could spare the patient from further outbreaks, such treatment could not only accelerate atherosclerosis process, but also increase the risk for cardiac decompensation. Hence, although the immediate course appears to be good, medium and long-term prognosis remain reserved.

  Acknowledgements Top

Conflicts of interest

There are no conflicts of interest.

  References Top

1.Gurler A, Boyvat A, Tursen U. Clinical manifestations of Behçet′s disease: an analysis of 2147 patients. Yonsei Med J 1997; 38:423-427.  Back to cited text no. 1
2. Kabbaj N, Benjelloun G, Gueddari FZ, Dafiri R, Imani F. Vascular involvements in Behçet′s disease. Based on 40 patient records. J Radiol 1993; 74:649-656.  Back to cited text no. 2
3. Bletery O, Mohatane A, Wechster B, Beaufils P, Valere P, Petit J, et al. Cardiac involvement in Behcet′s disease. Presse Med 1998; 17:2388-2391.  Back to cited text no. 3
4. Wechster B, Du LT, Kieffer E. Cardiovascular manifestations of Behcet′s disease. Ann Med Interne 1999; 150:542-554.  Back to cited text no. 4
5. Bowles CA, Nelson AM, Hammill SC, O′Duffy JD. Cardiac involvement in Behcet′s disease. Arthritis Rheum 1985; 28:345-348.  Back to cited text no. 5
6. Basaran Y, Degertekin M, Direskeneli H, Yakut C. Cardiac thrombosis in a patient with Behçet′s disease: two years follow-up. Int J Card Imaging 2000; 16:377-382.  Back to cited text no. 6
7. Iscan ZH, Vural KM, Bayazit M. Compelling nature of arterial manifestations in Behçet disease. J Vasc Surg 2005; 41:53-58.  Back to cited text no. 7
8. Ko G-Y, Byun JY, Choi BG, Cho SH. The vascular manifestations of Behçet′s disease: angiographic and CT findings. Br J Radiol 2000; 73:1270-1274.  Back to cited text no. 8
9. Hamza M. Maladie de Behçet. In: Kahn MF, Peltier AP, Meyer O, Piette JC, editors. Maladies et syndromes systémiques. 4th ed. Paris: Flammarion Médecine-Sciences; 2000. 883-924.  Back to cited text no. 9
10.Özoran K, Düzgün N, Gürler A, Yutkak H, Tokgöz G. Plasma von Willebrand factor, tissue plasminogen activator, plasminogen activator inhibitor, and antithrombin III levels in Behçet′s disease. Scand J Rheumatol 1995; 24:376-382.  Back to cited text no. 10
11.Hutchison SJ, Belch JJ. Behçet′s syndrome presenting as myocardial infarction with impaired blood fibrinolysis. Br Heart J 1984; 52:686-687.  Back to cited text no. 11
12.[No authors listed]. Criteria for diagnosis of Behçet′s disease. International Study Group for Behçet′s disease. Lancet 1990; 335:1078-1080.  Back to cited text no. 12
13.Drobinski G, Wechester B, Pavie A, Artigou JY, Marek P, Godeau P, Grosgogeat Y. Emergency percutaneous coronary dilation for acute myocardial infarction in Behçet′s disease. Eur Heart J 1987; 8:1133-1136.  Back to cited text no. 13
14.Tamura Y, Matsuoka A, Ohtaki E, Okabe M, Shibata A. Behçet′s disease complicated by acute myocardial infarction treated with percutaneous transluminal coronary angioplasty. Kokyu To Junkan 1988; 36:341-346.  Back to cited text no. 14
15.Erbilen E, Albayrak S, Gulcan E, Taser F, Bulur S, Ozhan H, Yazici M. Acute coronary stenosis in a young man with Behçet′s syndrome. Med Princ Pract 2008; 17:157-160.  Back to cited text no. 15
16.Tezcan H, Yavuz S, Fak AS, Aker U, Direskeneli H. Coronary stent implantation in Behçet′s disease. Clin Exp Rheumatol 2002; 20:704-706.  Back to cited text no. 16
17.Ando M, Kosakai Y, Okita Y, Nakano K, Kitamura S. Surgical treatment of Behçet′s disease involving aortic regurgitation. Ann Thorac Surg 1999; 68:2136-2140.  Back to cited text no. 17
18.Kosar F, Sahin I, Gullu H, Cehreli S. Acute myocardial infarction with normal corornary arteries in a young man with the Behçet′s disease. Int J Cardiol 2005; 99:355-357.  Back to cited text no. 18
19.Nurkalem Z, Uslu N, Gorgulu S, Eren M. Left main coronary thrombosis with essential thrombocythemia. J Thromb Thrombolysis 2006; 22:165-167.  Back to cited text no. 19
20.Ergelen M, Soylu O, Uyarel H, Yildirim A, Osmonov D, Orhan AL. Management of acute coronary syndrome in case of Behçet′s disease. Blood Coagul Fibrinolysis 2009; 20:715-718.  Back to cited text no. 20


  [Figure 1], [Figure 2], [Figure 3]

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