Cardiac amyloidosis (CA) is an infiltrative and restrictive cardiomyopathy that leads to heart failure, reduced quality of life, and death. The disease has two main subtypes, transthyretin cardiac amyloidosis (ATTR-CA) and immunoglobulin light chain cardiac amyloidosis (AL-CA), characterized by the nature of the infiltrating protein. ATTR-CA is further subdivided into wild-type (ATTRwt-CA) and variant (ATTRv-CA) based on the presence or absence of a mutation in the transthyretin gene. CA is significantly underdiagnosed and increasingly recognized as a cause of heart failure with preserved ejection fraction. Advances in diagnosis that employ nuclear scintigraphy to diagnose ATTR-CA without a biopsy and the emergence of effective treatments, including transthyretin stabilizers and silencers, have changed the landscape of this field and render early and accurate diagnosis critical. This review summarizes the epidemiology, pathophysiology, diagnosis, prognosis, and management of CA with an emphasis on the significance of recent developments and suggested future directions.

中文翻译：

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心脏淀粉样变性：被忽视，被低估并且可以治疗。

心脏淀粉样变性病（CA）是一种浸润性限制性心肌病，可导致心力衰竭，生活质量下降和死亡。该疾病有两种主要的亚型，转甲状腺素蛋白心脏淀粉样变性病（ATTR-CA）和免疫球蛋白轻链心脏淀粉样变性病（AL-CA），其特征在于浸润蛋白的性质。根据运甲状腺素蛋白基因中突变的存在与否，ATTR-CA可进一步分为野生型（ATTRwt-CA）和变异型（ATTRv-CA）。对CA的诊断不足，并逐渐被认为是保留射血分数的心力衰竭的原因。在不进行活检的情况下利用核闪烁显像技术诊断ATTR-CA的诊断进展以及出现了有效的治疗方法，包括运甲状腺素蛋白稳定剂和消音剂，已经改变了这一领域的面貌，使早期准确的诊断变得至关重要。这篇综述总结了CA的流行病学，病理生理学，诊断，预后和管理，重点是近期发展的意义和建议的未来方向。