bd is a multisystemic disease that may affect any organ in different combinations ? characterized by recurrent oral aphthous ulcers , genital ulcers , uveitis , and skin lesions .
involvement of the heart is called cardio - bd , which includes pericarditis , myocarditis , endocarditis , endomyocardial fibrosis , atrial fibrillation , ventricular arrhythmias , coronary arteritis , acute myocardial infarction , and dilated cardiomyopathy ( 1 ) .
we report a rare case of this syndrome , associated with aneurysm of the non - coronary sinuses of valsalva , resulting in symptomatic aortic valve regurgitation .
a 38-year - old man was referred for investigation of a 5-day history of paroxysmal nocturnal dyspnea in may 2008 .
he had a three - year history of recurrent genital ulceration initially diagnosed as genital herpes , but never confirmed on culture or nucleic acid amplification testing ; sequential treatment with aciclovir over a four - month period did not alleviate his genital symptoms . and
he had no history of cardiac or respiratory disease , nor any cardiac risk factors .
clinical examination revealed aphthous stomatitis , genital ulceration , erythema nodosum bilaterally and a grade 3/6 systolic murmur , audible at the 2nd intercostal space on the left sternal border .
hematologic examination showed that high sensitive c - reactive protein ( hs - crp ) was 105 mgl and erythrocyte sedimentation rate ( esr ) is 70 mmhr .
anti nuclear antibody ( ana ) , anti double strand dna antibody ( anti ds dna ) and muscle biopsy specimens for connective tissue disease were negative .
chest x - ray revealed cardiomegaly with a cardiothoracic ratio of 55% and a slightly widened mediastinum .
1 ) . the left ventricular ( lv ) end - diastolic was 58 mm , and the lv ejection fraction was 53% . based on these findings
, the diagnosis of aneurysm of the sinus of valsalva associated with bd was made . because the patient 's condition was in the active phase , prednisolone
was administered over one month until the inflammatory signs ( hscrp and esr ) became negative .
considered that our patient presented a competent valve and only one sinus involved , surgeon repaired the affected sinus only .
he was discharged without any complications on postoperative day 15 , and was doing well at five - year follow - up after the operation . showing non - coronary - sinus of valsalva aneurysm
aneurysms of the sinus of valsalva 's etiologies include atherosclerosis , infection such as syphilis , congenital disease such as marfan 's syndrome , trauma , and autoimmune disease such as bd .
bd is a chronic , relapsing multisystem vasculitis with predominant involvement of the oral and genital mucosa ( 1 , 2 ) .
bd has a worldwide distribution but is prevalent in japan , the middle east , and some mediterranean countries , and it often affects younger adults and is more common in men than women .
james et al . demonstrated that valsalva sinus aneurysm was the leading causes of death in patients with bd ( 3 ) . in our patient , echocardiography revealed aneurysm formation of the sinus of valsalva , and should bd is the etiology ?
the patient had a history of recurrent genital ulceration , on admission , clinical examination revealed aphthous stomatitis , genital ulceration , and erythema nodosum bilaterally , which could provide clues to differentiate diagnosis . if neglected these symptoms , it maybe at a loss of initial diagnosis of genital herpes .
in addition to recurrent genital ulceration , aphthous ulcers and skin lesion , a clinical diagnosis of bd should be made , because other examination showed that high crp and esr , and ana , anti dsdna and muscle biopsy specimens for connective tissue disease were negative .
aneurysms of the sinus of valsalva may incur complications if untreated , such as right ventricular outflow tract obstruction , coronary artery occlusion , aortic regurgitation and congestive heart failure , so surgical treatment should be considered ( 4 , 5 ) . but to aim directly at etiology is the most of all , and continuous steroid and immunosuppressive agent therapy are most important to maintain the integrity to prohibit recurrence of bd .
our patient was doing well at five - year follow - up after the operation .
it also highlights the importance of recognizing systemic disease and maintaining a holistic approach when treating patients .
 abstracta 38-year - old man with a history of recurrent genital ulceration initially diagnosed as genital herpes was admitted after presenting with paroxysmal nocturnal dyspnea . 
 echocardiography revealed aneurysm formation of the sinus of valsalva . on diagnosis of an aneurysm of the sinus of valsalva associated with behet 's disease 
 ( bd ) , surgeon repaired the affected sinus only , and continuous steroid therapy maintained the integrity . 
Introduction
Case Report
Discussion