Pathergy test strongly suggested Behcet's disease, which was the final diagnosis. Cross-sectional transthoracic echocar- diagram (TIE) demonstrated thrombi in ...
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Thorac. cardiovasc. Surgeon 46 (1998)
K. Kirali, A. Clvelek, B. Daglar. M. Sismanoqlu, E. Akinci, T. Berki,
O. lsik, and C. Yakut
Thorac. card iovasc. Surgeon 46 ( 1998) 102 - 105 © Georg Thi eme Verlag Stuttgart - New York
An Uncommon Complication of Behcet's Disease: Intracardiac Thrombosis Needing Surgical Treatment K. Kiroli. A. Cive/ek. B. Dog/or, M. $i~mo nog/u. E. Akinci,
T. Berki, 6. I~ik, ond C. Yokut Department of Cardiot horacic Surgery, kosuyolu Heart and Research Hospital. Istanbul, Turkey
Behcet's syndrome is a multisystem disorder presenting with recurrent oral and genital ulcerations as well as uveitis often leading to blindness. Although vascular lesions are a common complication of this disease. cardiac involvement is extremely rare. In this unusual case a young man had symptoms primarily
An Rare Complication of Behcet 's Disease: Intracardiac Thrombosis Needing Surgery
Thorae. cardiovase. Surgeon 46 (1998 ) 103
related to recurrent riqht-atrial andright-ventricularthrombi unresponsive to medical treatment. The intracardiac lesion was successfully treated by surgical excision with the use of cardiopulmonary bypass. Surgical treatment should be considered in Behcet's disease complicated by cardiac thrombi. when a thrombus recurs despite medical treatment. Key words: Behcet's disease - Intracardiac thrombosis
Introduction
Originally described as a clinical syndrome of recurrent oral and genital ulcers and relapsing iritis. Behcet 's disease is now recognized as a chronic multisystem illness involving many organs wit hin the body ( 1). The disease has a worldw ide distr ibution. but the prevalence is considera bly higher in Turkey. Israel. Leban on. Iran . and the Far East (japan, Korea, China ) than in the rest of the wo rld (2). It affects mainly young adults, wit h ma le patients experiencing a more severe clinical progress than female (2). Behcet's disease is considered an autoimmune disease because of the common denominator of vasculitis in most patients. Although supe rficial and deep peripheral vein thrombosis is seen in one-fourth of the patients, cardiac involvement is extremely rare (3). We present an unusual case of Behcet's disease in which the presenting symptoms were related to recurrent thrombi in right atrium and right ventricle.
Fig. 1 Preoperative echocardiographic view demonstrating thrombi in the right atrium and ventricle.
Case Report A28-yea r-o ld male ofTurkish nationality was ad mitted to hospital with a history of dyspn ea, neck swelling, and limitation in daily activities. The patient complained of recurrent painful ora l and genital ulcerations over a 2-year period. Erythrocyte sedimentation ratewas found to be 33 rnrn/hour, Rheumatoid factor was strongly positive.antinuclear antibody test was negat ive but ant i-DNA and HLA-BS tests were found to be positive. Pathe rgy test stro ngly suggested Behcet's disease, which was the final diagnosis. Cross-sectional transthoracic echocardiagram (TIE) dem onstrated t hrombi in the right atrium and right ventricle which extended to the inferior vena cava. Both CT scan and magnet ic resonance imaging (MRI) confirmed the diagnosis. The thrombi did not cause intracavitary obstruct ion andtherewas no history oft hromboembolism.The patient was placed on cyclosporin A, 3 mgjk g/day, met hylpredn isolone, 0.5 mg/kg/day, and anticoagulant treatm en t wit h warfarin (dose adjusted accor ding to INR), and aspirin, SOO mg/day. Complete lysis was achieved and after 3 month s TI E de monstrated no resid ua l th rombi. Two years later the patien t was readm itted to the hospital, complaining of shortness of breath. ascites, and peripheral edema.TIEwas repeated and right-atria l and right-ventricular thrombi were again demonstrated although he was receiving an anticoagulant (Fig. 1). The patient was referre d for open heart surgery. A median sternotomy was performed and standard cardiopulmonary techniques were instituted. Cardiopulmonary bypass was carried out by separate selective venous cannulation and ascending aorta. Following pericardiectomy, the exte rna l appea rance of the right atrium suggested a solid mass invading infer ior of th e right at rial free wa ll (Fig. 2 A). Following right atriotomy the right atrium was opened and ex-
Fig.:2 Intracardiac views. A) External appearance of the right atrium su ggested that a solid massinvaded right-atrial freewall. B)The massin the rightatrium extended throughthe tricuspi dvalveto the subva lvular space. leading to tricuspid valve stenosis. C) The resected area was repaired by gluteraldehyde-treated pericardial patch.
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Thorac. card iovas c. Surgeon 46 (199 8)
K. Kirali, A. Civelek, B. Daglar, M. Sisrnanoqlu. E. Akinci, T. Berki, 6. lsik, and C. Yakut
plored. There was a thrombus tightly adherent to the at rial free wall but the re wa s no freely float ing thrombus in the at rial cavity. The rig ht ventricle wa s explored via right ventriculotomy. The ma ss in the righ t atrium extended through the tricuspid valve into the subvalvu lar space, cau sing stenosis of the tricuspid valve (Fig. 2 B). The right-ventric ular outflow tract and pu l-
Fig.3 Resected thrombus specimen was dyed with hemotoxilin-eosi n stain. Inflammatory infiltrate part of thrombus organization and recanalized thrombus is seen around myocardial fibers (x 40).
Cardiac involvement in Behcet's disease is uncommon but such abnormalities as myoc ard itis , marasmic endocarditis, and coronary artery aneurysms have been described (4,6 ,8,9 ). Intracard iac thro mbi are extremely rare (3,4, 6,7,9 ). In a series of 137 patients wi th Behcet's disease, Kor;et al. des cri be d only one case of intracardiac thrombus wi t hi n t he right ven tri cle (4) . The pathologic mechanism of microvascular thrombus formation in vasculitis is be lieved to be due to endothelial cell ischaemia or disruption that leads to en hancemen t of platelet aggregation (10). Impai red fibrinolys is as a result of en dothelial cell injury from deposited immune complexes is anothe r possible mechanism. Prolonged euglobulin lysis times an d abnormal fibrin leve ls were found in several types of vasculitis, including Behcet's disease (11). Additionally, markedly suppressed release of vascu lar t issue plasminogen activato r (TPA) stores was reported in syste mic and cutaneous vasculitis (12). The mentioned abnorma lities most likely reflect endo t he lia l cell injury fro m vascular inflammatio n. Another possi ble patho logic mec hanism of thrombosis in Behcet's dise ase is at tribut ed to the presence of antiphospholipid antibodies, which are reported to be present in 18%of cases (13). Even though Mendes et al. had success fully surgically treated a right-ventricular thrombus in an unusual case of Behcet's disease (3) , two weeks later their patient returned with recurrent righ t-ventricular thrombus. After medical treatment complete lysis was achieved in that case. In our case me dica l treatment wa s origi na lly successful. Surgical treat men t became necessa ry w he n a recurrent thro mb us caused congestive heart failure whil e the patient was on medical treatment.
mona ry arte ry were free of disease. The invaded right-atria l free wall was resected leaving a small bridge between two separate areas. This area defect was repai red by glute raldehydet reated pe ricardial patch (Fig. 2 C). To clear the subvalvular space of thrombi we had to split the tricuspid posteroseptal commissure. The postoperative course was uneventful. TIE was repeated in the postoperative 10th month. There were no residual or recur rent th rombi. A m ild de gree of tricuspid insufficiency (1/4 degree ) was detected.
Early echocardiography seems advisable in patients with Behcet's disease to early detect any cardiac involvement, despite its rarity. First-line treatment is medical the rapy , but a thrombus can become massive and extensive, so that congestive heart failure, for example, may demand surgical measures. References
International Study Group for Behcet's Disease. Criteria for diagnosis of Behcet's Disease. La ncet. 1990;335 : 1078- 80. 2 Tunaci A, Berkmen YM, G6kmen E. Thoracic involvement in Behcet's Disease: Pathologic, clinical, and imaging features . Am ] Roent genol. 1995;164 :51-5. 3 Mendes LA, Magraw LL, Aldea GS, et al. Right ventricu lar th rombu s: An unusual manifestation of Behcet's disease.] Am Soc Echocardiogr.1994;7 :43 8 -40. 4 Koc Y, GOIIO I,Akpek G,et al.Vascular involvement in Behcet's dis. ease.] Rheumatol. 1992;19:402-10. 5 Lie ]. Vascular involvement in Behcet's disease: arterial and venous and vessels of all sizes.] Rheumatol. 1992;19: 341-2. 6 Lie J. Cardiac and pulmonary manifestations of Behcet's syndrome . Pathol Res Pract. 1988;183 :347 - 52. 7 Candan I, Erol C. Sonel A, Akalm H. Behcet's disease: cardiac and pulmonary involvement. Eur Heart J. 1986; 7 : 999 -1002. 8 Higashihara M, Mori M, Takeuchi A,et al. Myocarditis in Behcet's disease - a case report and review of the literatu re. ] Rheumato l. 1982:9 : 630-3. 9 Vanhaleweyk G, EI-Ramahi K, Hazmi M, et al. Right atrial, right vent ricular and left ventri cular thrombi in (incomplete) Behcet's disease. Eur Heart]. 1990:11 :957 - 9. 10 Shmitz-Haubne r U, Knop] . Eviden ce for an endo thelia l cell dysfunction in association with Behcet's disease. Thromb Res. 1984;34 : 277- 85. 1
The resected rig ht -atrial free wa ll was sent for pathologica l examination, with the res ults showing old and newly formed reca nalized thrombi, and some part of the specimen was infiltrated by polymorphonuclear leukocytes an d lymphocytes, which wa s thought to be part of the process of thrombus organization (Fig. 3 ). Discussion
Behcet's disease is now recognized as a multisystem illness in w hich t he in itia l man ifestations may not be confined to the classic symptom co mp lex of ora l and genital ulcerations and recurrent uveitis. Vascular involvement is frequen t and has been reported to occur in up to one third of patients (4 - 5 ). Venous lesions are the most common abnormality and usually consist of recurrent superficia l and deep thrombophelebitis, most often involving the lower extremities (4 - 5). Thrombosis of the vena cava is the second most common vascular lesion and often results in obstruction of the vessel (4) . Arterial thrombosis and spontaneous aneurysms, especially of the aorta and pu lmonary artery, are less common in Behcet's di sease but are responsib le for the majority of vasc ular dea ths (4 - 7).
Resection for CMV Ileiti s in a Patient Suppo rte d by a Lef t -Ventric ular Assist Device Aronowski E, Izak G. Decreased fibrinolysis in Behcet's disease. Thromb Diath Haemorrh. 1973;29 :610-8. 12 Jordan ]M , Allen NB, Pizzo 5V. Defective release of tissue plasmi-
11 Chajek T,
nogen activator in systemic and cutaneous vasculitis. AmJ Med. 1987;82 ; 397- 400. 13 Hull RG, Harris EN, Gharavi AE,et al. Anticardiolipin antibodies: Occurrence in Behcet's syndrome. Ann Rheum Dis. 1984:43 :7468.
Received /or Publication: November 12, 1997 Dr. Kaan Kiral i
Kosuyolu Kalp ve Arasn rma Hastanesi 81 020 Kadrkoy Istanbul Tilrkiye
Tho rac. cardiovasc. Surgeon 46 (1998)
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