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Version 1
Preserved in Portico This version is not peer-reviewed
Diagnostic Modalities in the Detection of Cardiac Amyloidosis
Version 1
: Received: 3 April 2024 / Approved: 8 April 2024 / Online: 9 April 2024 (09:32:10 CEST)
A peer-reviewed article of this Preprint also exists.
Bukhari, S.; Bashir, Z. Diagnostic Modalities in the Detection of Cardiac Amyloidosis. J. Clin. Med. 2024, 13, 4075. Bukhari, S.; Bashir, Z. Diagnostic Modalities in the Detection of Cardiac Amyloidosis. J. Clin. Med. 2024, 13, 4075.
Abstract
Cardiac amyloidosis (CA) results mainly from infiltration of myocardium by either immunoglobulin light chain fibrils (AL) or transthyretin fibrils (ATTR), causing restrictive cardiomyopathy and eventually death if untreated (3). AL derives from monoclonal immunoglobulin light chain produced by plasma cell clone in the bone marrow, while ATTR is the misfolded form of hepatically-derived transthyretin (TTR) protein, and could be hereditary (hATTR) or wild-type (wtATTR). Over the last decade, improvement in diagnostic imaging and better clinical awareness has unleashed a notable presence of CA in the community, especially ATTR in the elderly population. These multimodality imaging modalities include echocardiography, cardiac magnetic resonance and radionuclide scintigraphy with bone-avid tracers. There has been remarkable progress in the therapeutic landscape as well, and there are disease-modifying therapies available now that can alter the course of the disease and improve survival if initiated at an early stage in the disease. There remains an unmet need for detecting this disease accurately and early, so that these patients could benefit the most from newly-emerging therapies.
Keywords
Cardiac amyloidosis; transthyretin; echocardiography; cardiac magnetic resonance; Tc-pyrophosphate scintigraphy
Subject
Medicine and Pharmacology, Clinical Medicine
Copyright: This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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