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.

中文翻译：

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埃尔德海姆-切斯特病的心血管表现：两个病例的叙述性回顾

埃尔德海姆-切斯特病 (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，导致骨硬化性骨病变、主动脉周围包绕（“涂层”主动脉）、累及肾脏和输尿管的腹膜后纤维化（“毛状”）肾'），以及中枢神经系统的浸润。高达 70% 的患者可能会出现心血管受累，通常在计算机断层扫描/磁共振成像评估过程中发现。当出现时，心血管症状的表现可能有很大差异，从无症状到心包炎、致命性心包填塞、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 可累及大中型动脉，通常表现为动脉周围增厚（通常覆盖主动脉）并伴有狭窄/闭塞。尽管更多的心血管 ECD 病例已开始在文献中发表，但仍需要更多关于这些患者的结果以及心血管表现对 ECD 治疗的反应的数据。