Department of Cardiology, Smidt Heart Institute, Cedars-Sinai, Los Angeles, CA, USA.
, Los Angeles, USA.
Curr Oncol Rep. 2021 Mar 16;23(4):47. doi: 10.1007/s11912-021-01028-8.
Cardiac amyloidosis is an underrecognized cause of heart failure. We review clinical clues to the diagnoses, a rational approach to testing, and current and emerging therapies.
Advances in the diagnosis of amyloid cardiomyopathy include (1) use of technetium (Tc) bone-avid compounds which allow accurate noninvasive diagnosis of transthyretin cardiac amyloidosis (ATTR-CM) in the context of a negative monoclonal light chain screen; and (2) the use of serum and urine immunofixation electrophoresis with serum free light chains as an accurate first diagnostic step for light chain cardiac amyloidosis (AL-CM). Advances in treatment include tafamidis for ATTR-CM and immunologic therapies for AL-CM. With the advent of accurate noninvasive diagnostic modalities and effective therapies, early recognition of cardiac amyloidosis is paramount to implement a diagnostic algorithm and expeditiously institute effective therapies to minimize morbidity and mortality.
心脏淀粉样变是心力衰竭的一个被低估的病因。我们综述了心脏淀粉样变的诊断线索、合理的检查方法以及现有和新出现的治疗方法。
淀粉样心肌病的诊断进展包括：(1) 使用锝（Tc）骨亲和化合物，在单克隆轻链检测阴性的情况下，可准确进行转甲状腺素蛋白心脏淀粉样变（ATTR-CM）的非侵入性诊断；(2) 血清游离轻链免疫固定电泳在诊断轻链心脏淀粉样变（AL-CM）时作为一种准确的初始诊断步骤。治疗进展包括 tafamidis 治疗ATTR-CM 和免疫疗法治疗 AL-CM。随着准确的非侵入性诊断方式和有效的治疗方法的出现，早期识别心脏淀粉样变对于实施诊断方案和迅速采用有效的治疗方法以降低发病率和死亡率至关重要。
