Chiari Ermanna, Fracassi Francesco, D'Aloia Antonio, Vizzardi Enrico, Zanini Gregoriana, Rocca Patrizia, Metra Marco, Cas Livio Dei
Section of Cardiovascular Disease, Department of Applied Experimental Medicine, Brescia Study University, Brescia, Italy.
J Am Soc Echocardiogr. 2008 Sep;21(9):1079. doi: 10.1016/j.echo.2007.10.012. Epub 2008 Jan 15.
Behçet's disease (BD) is a multisystemic, chronic, inflammatory disease of unknown origin characterized by alternation of exacerbation and quiescence phases. Cardiac involvement in BD is infrequent. We report a case of a young man with BD with right ventricular thrombus and pulmonary thromboembolism. A 20-year-old man was admitted to our hospital with a 6-month history of dyspnea at rest, asthenia, and fever. Transthoracic echocardiography showed right wall thickened and presence of floating masses in the right outflow tract. Transesophageal echocardiography confirmed the presence of diffuse thrombosis in the right ventricle, with mobile ramifications in its outflow tract. Cardiovascular magnetic resonance and computed tomography of heart confirmed the presence of thrombi, the increased thickness of endocardiac tissue, and altered cardiac wall signal transmission. Computed tomography scan showed multiple pulmonary thrombi. Myocardial biopsy specimen showed diffused subendocardial thrombosis with damage of cardiac myocytes and presence of granulocytes. Six months after discharge, no cardiac masses were detected by transthoracic echocardiography. However, a few weeks after this last echocardiogram, the patient was again hospitalized for a new episode of acute pulmonary embolism. The patient was discharged with increasing dose of oral anticoagulant, with no evidence of cardiac masses at transthoracic echocardiography and thrombosis at computed tomography. The patient is still healthy on anticoagulant, immunosuppressive, and steroid therapy. This case provides a rare example of BD, in which we found-at the same time-heart and pulmonary manifestations, with the presence of right ventricular thrombus and pulmonary thrombi in situ.
白塞病(BD)是一种多系统慢性炎症性疾病,病因不明,其特点是病情有加重期与缓解期的交替。白塞病累及心脏的情况并不常见。我们报告一例患有白塞病的年轻男性病例,该患者出现右心室血栓和肺血栓栓塞。一名20岁男性因有6个月的静息时呼吸困难、乏力和发热病史而入住我院。经胸超声心动图显示右心室壁增厚,右心室流出道有漂浮物。经食管超声心动图证实右心室存在弥漫性血栓形成,其流出道有活动分支。心脏磁共振成像和心脏计算机断层扫描证实存在血栓、心内膜组织增厚以及心脏壁信号传导改变。计算机断层扫描显示多发性肺血栓。心肌活检标本显示弥漫性心内膜下血栓形成,伴有心肌细胞损伤和粒细胞存在。出院6个月后,经胸超声心动图未检测到心脏肿物。然而,在最后一次超声心动图检查几周后,患者因新的急性肺栓塞发作再次住院。患者出院时口服抗凝剂剂量增加,经胸超声心动图未发现心脏肿物,计算机断层扫描也未发现血栓形成。患者在接受抗凝、免疫抑制和类固醇治疗后仍保持健康。该病例提供了一个罕见的白塞病实例,在此病例中我们同时发现了心脏和肺部表现,以及原位存在的右心室血栓和肺血栓。