心脏淀粉样变的研究进展  

Research Advances in Cardiac Amyloidosis

作　　者：张昕彤 任卫东[1] 张晶[1] 谭雪莹 ZHANG Xintong;REN Weidong;ZHANG Jing;TAN Xueying(Department of Ultrasound,Shengjing Hospital,China Medical University,Shenyang 110004,China)

机构地区：[1]中国医科大学附属盛京医院超声科

出　　处：《中国医科大学学报》2019年第11期1029-1032,1036,共5页Journal of China Medical University

基　　金：国家自然科学基金(81571686)

摘　　要：心脏淀粉样变是淀粉样蛋白沉积在心脏引起的疾病,其发病率低,预后极差,临床表现为限制性心肌病或难治性心力衰竭。淀粉样蛋白纤维在细胞外间质中弥漫沉积,包绕心肌细胞。心室和心房均受累,心脏收缩、舒张功能减低,并可引发心律失常。心脏淀粉样变的诊断方法包括心电图、超声心动图等影像学手段、实验室检查以及组织活检。各种不断更新完善的诊断手段协助早期诊断心脏淀粉样变,评估临床预后,有利于尽早制定治疗计划,改善患者生活质量。本文总结了心脏淀粉样变性的主要亚型、诊断方法及治疗与预后的最新研究进展。Cardiac amyloidosis(CA) is a rare disease with cardiac tissue deposition of amyloid. Clinical symptoms are marked with restrictive cardiomyopathy and refractory heart failure. Its incidence is low and prognosis is usually poor. Amyloid fibrils deposit in the extracellular interstitium diffusively among cardiomyocytes. Both the ventricles and atria are infiltrated,which causes systolic and diastolic dysfunctions and arrhythmia. Diagnostic approaches include electrocardiography,imaging modalities such as echocardiography,laboratory tests,and tissue biopsy. Early diagnosis of cardiac amyloidosis is assisted by various innovative and advanced diagnostic methods,which helps in evaluating prognosis,deciding therapies,and improving patient quality of life. Here,we summarize the main subtypes and research advances in the diagnostic methods,treatments,and prognosis of cardiac amyloidosis.

关 键 词：心脏淀粉样变 淀粉样蛋白 诊断 治疗 

分 类 号：R541[医药卫生—心血管疾病]