Cardiac Amyloidosis: Evolving Diagnosis and Management: A Scientific Statement From the American Heart Association
Cardiac Amyloidosis: Evolving Diagnosis and Management: A Scientific Statement From the American Heart Association
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DOI：
10.1161/cir.0000000000000792
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发表时间：
2020-07-07
期刊：
影响因子：
37.8
通讯作者：
Ruberg, Frederick L.
中科院分区：
文献类型：
--
作者：
Kittleson, Michelle M.;Maurer, Mathew S.;Ruberg, Frederick L.
转甲状腺素淀粉样心肌病（atr - cm）是一种由转甲状腺素细胞外沉积引起的限制性心肌病，转甲状腺素通常参与甲状腺素和视黄醇结合蛋白在心肌中的运输。对atr - cm的热情随着3个同步领域的进步而增长：成像技术允许对atr - cm进行准确的无创诊断，而无需进行确认性心内膜肌活检；观察性研究表明，atr - cm的诊断在很大一部分心力衰竭患者中可能被低估；在阐明淀粉样蛋白形成机制的基础上，现已批准治疗atr - cm的疗法。由于atr - cm的治疗在明显的心功能障碍之前给予治疗可能是最有效的，因此使用易于获得的无创检查来早期识别受影响的个体是至关重要的。本科学声明旨在指导临床实践，并通过涵盖当前的诊断和治疗策略，以及atr - cm未满足的需求和积极调查领域，促进管理一致性。
Transthyretin amyloid cardiomyopathy (ATTR-CM) results in a restrictive cardiomyopathy caused by extracellular deposition of transthyretin, normally involved in the transportation of the hormone thyroxine and retinol-binding protein, in the myocardium. Enthusiasm about ATTR-CM has grown as a result of 3 simultaneous areas of advancement: Imaging techniques allow accurate noninvasive diagnosis of ATTR-CM without the need for confirmatory endomyocardial biopsies; observational studies indicate that the diagnosis of ATTR-CM may be underrecognized in a significant proportion of patients with heart failure; and on the basis of elucidation of the mechanisms of amyloid formation, therapies are now approved for treatment of ATTR-CM. Because therapy for ATTR-CM may be most effective when administered before significant cardiac dysfunction, early identification of affected individuals with readily available noninvasive tests is essential. This scientific statement is intended to guide clinical practice and to facilitate management conformity by covering current diagnostic and treatment strategies, as well as unmet needs and areas of active investigation in ATTR-CM.