Abstract：
Erdheim-Chester disease (ECD) is a rare \'L\' (Langerhans) group histiocytic neoplasm that affects a multitude of organ systems, causing osteosclerotic bone lesions, periaortic encasement (\'coated\' aorta), retroperitoneal fibrosis involving kidneys and ureters (\'hairy kidney\'), and infiltration of the central nervous system. Cardiovascular involvement can occur in up to 70% of patients and is usually found during computed tomography/magnetic resonance imaging evaluation. When present, cardiovascular symptoms can have wide variability in presentation from asymptomatic to pericarditis, fatal cardiac tamponade, myocardial infarction, conduction abnormalities, heart failure, renal artery stenosis, and claudication. Cardiac involvement found on imaging includes right atrial pseudotumor, right atrioventricular groove infiltration, and pericardial effusions. ECD can involve the large- and medium-sized arteries, often seen as periarterial thickening (commonly coating the aorta) with stenosis/occlusion. Although more cardiovascular ECD cases have begun to be published in the literature, more data are needed on the outcomes of these patients, as well as how cardiovascular manifestations respond to treatment of ECD.
摘要：
Erdheim-Chester病（ECD）是一种罕见的L（朗格汉斯）组组织细胞肿瘤，影响多个器官系统，引起骨硬化性骨病变，主动脉周围包裹(主动脉涂层),涉及肾脏和输尿管的腹膜后纤维化（“毛肾”），和中枢神经系统的渗透。多达70％的患者可发生心血管受累，通常在计算机断层扫描/磁共振成像评估期间发现。当存在时,从无症状到心包炎，心血管症状可以具有广泛的变异性，致命的心脏填塞,心肌梗塞,传导异常,心力衰竭,肾动脉狭窄,和跛行。影像学发现的心脏受累包括右心房假瘤，右房室沟浸润,和心包积液.ECD可以累及大中型动脉，通常被视为动脉周围增厚（通常覆盖主动脉）伴有狭窄/闭塞。尽管更多的心血管ECD病例已经开始在文献中发表，这些患者的结局需要更多的数据，以及心血管表现对ECD治疗的反应。
