Department of Cardiology, Expert Center for Rare Genetic Cardiovascular Diseases, Emergency Institute for Cardiovascular Diseases "Prof. Dr. C. C. Iliescu", Sos. Fundeni 258, 022322 Bucharest, Romania.
University of Medicine and Pharmacy "Carol Davila", Euroecolab, Bucharest, Romania.
Eur Heart J Cardiovasc Imaging. 2020 Aug 1;21(8):833-844. doi: 10.1093/ehjci/jeaa063.
Amyloidosis is a systemic infiltrative disease, in which unstable proteins misfold, form aggregates and amyloid fibrils which can deposit in various organs: heart, kidneys, liver, gastrointestinal tract, nervous system structures, lungs, or soft tissue. Cardiac amyloidosis (CA) diagnosis requires awareness, high level of clinical suspicion and expertise in integrating clinical, electrocardiographic, and multimodality imaging data. The overall scenario is complex and no single test emerges over the others, but different techniques are useful at various stages of the diagnostic workup. After a clinical suspicion of CA is raised by various non-imaging red-flags, eligible patients should undergo complete echocardiography and multiparametric cardiovascular magnetic resonance imaging. Even though the clinical suspicion of CA is confirmed by cardiac imaging, the accurate differentiation between the two most frequent and treatable amyloid types, i.e. light chain (AL) and transthyretin (ATTR) requires further work-up including phosphate scintigraphy. This article reviews the latest and essential data on multimodality imaging of patients with suspected or confirmed CA in a useful and practical manner for the general and imaging cardiologists.
淀粉样变性是一种全身性浸润性疾病,其中不稳定的蛋白质错误折叠,形成聚集体和淀粉样纤维,可沉积在各种器官中:心脏、肾脏、肝脏、胃肠道、神经系统结构、肺或软组织。心脏淀粉样变性(CA)的诊断需要有认识、高度的临床怀疑和整合临床、心电图和多模态成像数据的专业知识。整体情况较为复杂,没有一种单一的检查方法优于其他方法,但不同的技术在诊断工作的不同阶段都有一定的作用。在通过各种非成像的“红色标记”提示临床疑似 CA 后,应让合适的患者接受完整的超声心动图和多参数心血管磁共振成像检查。即使心脏成像证实了 CA 的临床怀疑,但要准确区分两种最常见且可治疗的淀粉样变类型,即轻链(AL)和转甲状腺素(ATTR),还需要进一步检查,包括磷闪烁显像。本文以一种对普通心脏病专家和影像学专家都有用且实用的方式,对疑似或确诊 CA 患者的多模态成像的最新和重要数据进行了综述。