心脏磁共振在AL型及ATTR型心肌淀粉样变中的应用进展

The advances in cardiovascular magnetic resonance imaging for light-chain and transthyretin-related amyloidosis

淀粉样变性是一类系统性疾病,其特征是不同来源的错误折叠蛋白片段在器官内沉积。淀粉样变性可累及心脏,称为心肌淀粉样变,且病变的严重程度是决定患者预后的主要因素。目前临床最常见的心肌淀粉样变类型主要为原发性免疫球蛋白轻链型(light-chain amyloidosis,AL)和遗传性甲状腺素转载蛋白型淀粉样变(transthyretinrelated amyloidosis,ATTR),而不同类型的淀粉样变的治疗手段及预后也不相同。因此,早期、准确诊断心肌淀粉样变尤为重要。心脏磁共振(cardiovascular magnetic resonance,CMR)具有多模态、多参数成像特点,不仅能早期发现心肌淀粉样变的形态及功能异常,还能准确评判心肌组织学特征及微循环改变,为疾病的诊断、危险分层及预后评估提供重要信息。笔者将对心肌淀粉样变性的CMR应用进展进行综述。

Amyloidosis represents a group of systemic diseases characterized by deposition of diverse origins misfolded protein fragments in multiple organs.Cardiac involvement,termed cardiac amyloidosis(CA),is the major factor determining the prognosis of patients.The most common types of cardiac amyloidosis in the clinic are light-chain amyloidosis(AL)and transthyretin-related amyloidosis(ATTR).The treatment and prognosis vary according to different amyloidosis types.Therefore,early and accurate diagnosis is essential in clinical setting.Cardiovascular magnetic resonance(CMR)characterized by multi-modality and multiparameter imaging,could early detect the morphological and functional abnormalities and accurately evaluate the histological feature and microvascular changes in cardiac amyloidosis,providing important information for the diagnosis,risk stratification and prognosis.This article will review the progress of CMR application in cardiac amyloidosis.