庭山 博行, 近藤 信介, 宮沢 幸世, 西本 良博, 増田 義昭, 稲垣 義明

Hiroyuki NIWAYAMA, Nobusuke KONDO, Yukiyo MIYAZAWA, Yoshihiro NISHIMOTO, Yoshiaki MASUDA, Yoshiaki INAGAKI

千葉大学医学部第3内科

The 3rd Dept. of Internal Medicine, Chiba University School of Medicine

キーワード : Behçet's disease, Cardio-Behçet disease, Echocardiography, Right ventricular thrombus, Aneurysm of the sinus of Valsalva

Although 8% of patients with Behçet's disease have vascular complications, cardiac involvement in Behçet's disease is very rare. Only a few cases of valvular disease, myocarditis, endocarditis, pericarditis, or coronary artery disease have been reported in conjunction with Behçet's disease. We report here 2 cases of cardio-Behçet's disease. The first case is a 43-year-old man who presented with the classic form of Behçet's disease. 2-D echocardiography and right ventriculography showed right atrial and ventricular dilatation with tricuspid regurgitation and mobile thrombus in the right ventricular apex. Lung per fusion scintigraphy showed multiple per fusion defects in the lung increasing on sequential yearly exams that were not present on his first admission. The second case is a 45-year-old woman with incomplete Behçet's disease found to have a systolic and diastolic heart murmur. 2-D echocardiography showed an aneurysm of the noncoronary sinus of Valsalva which projected into the left atrium and ventricle. Doppler echocardiography showed severe aortic regurgitation and mild mitral regurgitation. These findings were confirmed by aortography. During surgery a rupture of the aneurysm was found. In the first case the right ventricular thrombus is thought to have been caused by endocarditis, although histopathological confirmation is lacking. Candan et al reported a case of Behçet's disease with pulmonary infarction due to releases of a right ventricular thrombus in which the existence of endocarditis was confirmed by biopsy. Only 4 cases of Behçet's disease have been reported in which aortic regurgitation was accompanied by an aneurysm of the sinus of Valsalva similar to our second case. Cardio-Behçet's disease is often lethal, so echocardiography is necessary to follow the course of the disease and useful in diagnosing cardiac complications.