Halatchev Ilia G, Zheng Jingsheng, Ou Jiafu
Division of Cardiology, John Cochran Veterans Affairs Medical Center, St. Louis, USA.
Division of Cardiology, Washington University School of Medicine, St. Louis, MO, USA.
J Thorac Dis. 2018 Mar;10(3):2034-2045. doi: 10.21037/jtd.2018.03.134.
Cardiac amyloidosis is thought to be a rare group of diseases caused by extracellular deposition of misfolded proteins in the extracellular cardiac matrix resulting in heart failure with preserved ejection fraction (HFpEF). This review focuses on the similarities and differences between the pathophysiology, clinical presentation and diagnostic tests of wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) compared to immunoglobulin light chain amyloidosis and hereditary cardiac amyloidosis. We address some obstacles to timely diagnosis and opportunities for management of the clinical symptoms as well as possibility of future novel disease modifying therapies.
心脏淀粉样变性被认为是一组罕见的疾病,由错误折叠的蛋白质在细胞外心脏基质中沉积引起,导致射血分数保留的心力衰竭(HFpEF)。本综述重点关注野生型转甲状腺素蛋白心脏淀粉样变性(ATTRwt-CA)与免疫球蛋白轻链淀粉样变性和遗传性心脏淀粉样变性在病理生理学、临床表现和诊断测试方面的异同。我们探讨了及时诊断的一些障碍、管理临床症状的机会以及未来新型疾病修饰疗法的可能性。