解缠结淀粉样变性:心脏淀粉样变性的最新进展。

International Journal of Heart Failure Pub Date : 2020-07-31 eCollection Date: 2020-10-01 DOI:10.36628/ijhf.2020.0016
Darae Kim, Jin-Oh Choi, Kihyun Kim, Seok Jin Kim, Eun-Seok Jeon
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引用次数: 9

摘要

心脏淀粉样变性(CA)是一种高度未被诊断的心力衰竭病因。淀粉样蛋白轻链(AL)和淀粉样转甲状腺素(ATTR)型心肌病是心脏淀粉样蛋白的两种主要亚型。淀粉样蛋白纤维沉积通过机械浸润心肌或直接的心脏毒性引起心功能障碍。及时诊断对于启动改善疾病的治疗和提高CA患者的生存率非常重要。因此,在评估心力衰竭患者时,医生必须意识到“危险信号症状”,这些症状会增加对CA的怀疑。虽然心内膜活检是一种明确的诊断工具,但随着近年来无创成像技术的进步,非活检诊断在ATTR型CA中是可行的。AL和ATTR型CA的治疗都取得了重大进展,CA的生存率也有所提高。除了心力衰竭的一般治疗外,AL和ATTR型CA的治疗选择也在增加。鉴于淀粉样蛋白的全身性,多学科团队方法对CA的治疗至关重要。随着AL和ATTR型淀粉样蛋白病的诊断和治疗方案的最新发展,它不再被认为是一种不可治疗的疾病。
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Untangling Amyloidosis: Recent Advances in Cardiac Amyloidosis.

Cardiac amyloidosis (CA) is a highly underdiagnosed cause of heart failure. Amyloid light-chain (AL) and amyloid transthyretin (ATTR) cardiomyopathy are two major subtypes of cardiac amyloid. Amyloid fibril deposits cause cardiac dysfunction by mechanically infiltrating the myocardium or by direct cardiotoxicity. Achieving a timely diagnosis is important to initiate disease-modifying therapies and improve the survival of patients with CA. Therefore, physicians must be aware of "red flag symptoms" that increase suspicions for CA when assessing heart failure patients. Although endomyocardial biopsy is a definitive diagnostic tool, with recent advances in non-invasive imaging, non-biopsy diagnosis is feasible in ATTR CA. There have been major advances in treatments for both AL and ATTR CA, and survival of CA has improved. In addition to general management of heart failure, numerous treatment options are increasing for both AL and ATTR CA. Given the systemic nature of amyloids, multi-disciplined team approaches are crucial to management of CA. With recent development of diagnosis and treatment options for both AL and ATTR amyloidosis, it is no longer considered a non-treatable disease.

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