behet 's disease ( bd ) is characterized by recurrent aphthous stomatitis , genital ulcers , and various skin lesions .
bd can also involve other systems such as ocular , gastrointestinal , articular , neurological and cardiovascular systems .
the frequency of cardiovascular involvement is estimated to be 4 - 46%.1)2 ) common vascular manifestations are thrombophlebitis and arteritis , which occur in as many as one - third of the patients.3 ) aseptic endocarditis is a rare manifestation of bd and is mostly found in the form of intracardiac thrombus or endomyocardial fibrosis.3)4 ) nonrheumatic tricuspid valve stenosis ( ts ) is extremely rare , and to our knowledge , it has never been reported in the english literature in patients with bd .
we describe a case of a female bd patient with aseptic tricuspid valve ( tv ) endocarditis presenting as ts .
a 39-year - old female was admitted to kyungpook national university hospital with a 3-month history of dyspnea on exertion and abdominal distension .
signs of right heart failure such as pitting edema , palpable liver and neck vein distension were noted on physical examination . she had been diagnosed with bd four years ago .
although her previous clinical courses fluctuated , she did not show any signs or symptoms of bd since one year before admission . at the time of admission , evidence of bd disease exacerbation
was absent ; esr 20 mm / hr ( reference 0 - 20 mm / hr ) , ferritin 71.50 ng / ml ( reference 13 - 150 ng / ml ) .
transthoracic echocardiography ( tte ) showed normal left ventricular function , normal aortic and mitral valve function , morphology , and a moderate to large amount of pericardial effusion .
however , precise evaluation of right heart was difficult on tte due to poor echo window .
transesophageal echocardiography showed severe ts with an ill - margined echogenic mass , and a mild to moderate amount of pericardial effusion ( figs . 1 and 2 ) .
any other possible causes of ts , such as cardiac tumors , carcinoid syndrome , marantic endocarditis , and wegener 's granulomatosis were not detected .
symptoms of right heart failure gradually progressed despite the appropriate steroid and immunosuppressive therapy.5 ) she was operated for ts .
the thickened tv was removed and was replaced with an artificial valve ( edwards - mira 31 mm ) .
pathologic examination showed valvulitis consisting of fibrinoid necrotic material and inflammatory cells ( figs . 3 and 4 )
these pathologic findings were consistent with those of previous reports presenting aseptic endocarditis in bd.6)7 ) after the tv replacement , she remained free from symptoms of right heart failure with immunosuppressive therapy and anticoagulant therapy .
endocarditis in bd may be limited to the valve leaflets or may spread to the ventricular or atrial wall and can result in serious complications , such as valvulopathy , organized thrombus or endomyocardial fibrosis.3)4)9 ) however , most cases of endocarditis in bd were detected in the form of organized thrombus or endomyocardial fibrosis.3)4 ) not only an intracardiac thrombus but also a massive endomyocardial fibrosis could cause serious functional obstruction of the tv.3)4)9 ) however , overt ts due to valvulitis has not been reported , even though the affected valve leaflets could either be thickened or replaced by fibrous tissues . also , we could not find any evidence of other combined sequelae of endocarditis , such as intracardiac thrombosis , endomyocardial fibrosis or valvulopathy of other valves .
mcdonald et al.10 ) reported for the first time , a case of endocarditis involving the normal mitral and aortic valves in a patient with bd .
histologic examination of these valves showed mononuclear infiltration with a few polymorphonuclear leukocytes and no fibrin deposits .
madanat et al.6 ) reported a case of bd and endocarditis with left atrial thrombosis mimicking myxoma .
postoperative pathologic examination revealed yellowish valvulitis involving the mitral valve leaflet with deep ulcerations on the valve surface , covered by fibrinous and necrotic masses with a significant growth of granulation tissue .
in other case reports of endocarditis in bd , several granulomas were found within the central portion of the vegetations and polymorphonuclear cells and lymphocytes infiltrated the small vessels near the vegetations.7 ) in our case , the pathologic examination showed vegetations , consisting of fibrinoid necrotic material , granulation tissue , and inflammatory cells , which were predominantly mononuclear cells .
therefore , ts was caused due to valvulitis as a possible sequelae of endocarditis in bd .
as in this case , the cardiac lesions in bd might progress insidiously in the absence of concurrent signs or symptoms of bd , or they were even diagnosed at autopsy.10)11 ) therefore , it might be difficult to detect endocarditis in the early stage .
these findings suggest that screening for cardiac involvement is required for early detection of endocarditis or other heart diseases in patients with bd .
 aseptic endocarditis is an uncommon complication of behet 's disease ( bd ) . 
 we describe a rare case of a 39-year - old female who had bd with aseptic endocarditis of the tricuspid valve ( tv ) presenting as tricuspid stenosis . 
 she was diagnosed with bd four years ago . 
 the mucocutaneous lesions were well - controlled with colchicine and short courses of corticosteroids . 
 she remained free of signs and symptoms of bd for one year without any medication . 
 three months before admission , she gradually developed dyspnea on exertion and peripheral edema . 
 echocardiography revealed dilated right atrium and markedly thickened tv with severe stenosis . 
 tv replacement was performed . 
 pathologic examination of the valve showed fibrinoid necrotic material and inflammatory cell infiltration . 
 blood cultures and cultures of the excised valve were negative for microorganisms . 