作者: Ana Martinez-Naharro , A. John Baksi , Philip N. Hawkins , Marianna Fontana
DOI: 10.1038/S41569-020-0334-7
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摘要: Systemic amyloidosis encompasses a debilitating, under-diagnosed but increasingly recognized group of disorders characterized by the extracellular deposition misfolded proteins in one or more organs. Cardiac amyloid leads to an infiltrative restrictive cardiomyopathy and is major contributor poor prognosis patients with systemic amyloidosis. In total, >30 can form fibrils, two main types that infiltrate heart are monoclonal immunoglobulin light-chain transthyretin amyloid. be acquired older individuals inherited from birth. Given nonspecific symptoms these disorders, high index suspicion paramount making correct diagnosis, which involve use non-invasive imaging methods such as echocardiography, bone scintigraphy cardiovascular MRI. past decade, MRI tissue characterization diagnose cardiac has revolutionized our understanding disease, leading changes patient care. However, need remains for improved awareness expertise, greater clinical suspicion, because initial clues provided electrocardiography echocardiography might not typical. With specific treatments now available, timely diagnosis important than ever. this Review, we discuss current novel approaches diagnostic