1 Introduction Cardiac amyloid(CA)is characterized by inexorably progressive heart failure making early diagnosis and treatment imperative.This article will review the pathophysiology,clinical presentation,diagnosis,p...1 Introduction Cardiac amyloid(CA)is characterized by inexorably progressive heart failure making early diagnosis and treatment imperative.This article will review the pathophysiology,clinical presentation,diagnosis,prognostication and treatment of patients with CA.2 Pathophysiology CA is the result of extracellular deposition of a misfolded protein into cardiac tissue,forming insoluble aggregations of rigid,nonbranching 10 nm wide fibrils.This causes impaired cardiac function by disrupting cardiac architecture,direct myotoxicity and ischemic injury secondary to infiltration of intramyocardial vessels.展开更多
文摘1 Introduction Cardiac amyloid(CA)is characterized by inexorably progressive heart failure making early diagnosis and treatment imperative.This article will review the pathophysiology,clinical presentation,diagnosis,prognostication and treatment of patients with CA.2 Pathophysiology CA is the result of extracellular deposition of a misfolded protein into cardiac tissue,forming insoluble aggregations of rigid,nonbranching 10 nm wide fibrils.This causes impaired cardiac function by disrupting cardiac architecture,direct myotoxicity and ischemic injury secondary to infiltration of intramyocardial vessels.
