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      Nuclear Imaging for the Diagnosis of Cardiac Amyloidosis in 2021

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          Abstract

          Cardiac amyloidosis is caused by the deposition of misfolded protein fibrils into the extracellular space of the heart. The diagnosis of cardiac amyloidosis remains challenging because of the heterogeneous manifestations of the disease. There are many different types of amyloidosis with light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis being the most common types of cardiac amyloidosis. Endomyocardial biopsy is considered the gold standard for diagnosing cardiac amyloidosis and differentiating amyloid subtypes, but its use is limited because of the invasive nature of the procedure, with risks for complications and the need for specialized training and centers to perform the procedure. Radionuclide cardiac imaging has recently become the most commonly performed test for the diagnosis of ATTR amyloidosis but is of limited value for the diagnosis of AL amyloidosis. Positron emission tomography has been increasingly used for the diagnosis of cardiac amyloidosis and its applications are expected to expand in the future. Imaging protocols are under refinement to achieve better quantification of the disease burden and prediction of prognosis.

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          Most cited references80

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          Tafamidis Treatment for Patients with Transthyretin Amyloid Cardiomyopathy

          Transthyretin amyloid cardiomyopathy is caused by the deposition of transthyretin amyloid fibrils in the myocardium. The deposition occurs when wild-type or variant transthyretin becomes unstable and misfolds. Tafamidis binds to transthyretin, preventing tetramer dissociation and amyloidogenesis.
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            Nonbiopsy Diagnosis of Cardiac Transthyretin Amyloidosis.

            Cardiac transthyretin (ATTR) amyloidosis is a progressive and fatal cardiomyopathy for which several promising therapies are in development. The diagnosis is frequently delayed or missed because of the limited specificity of echocardiography and the traditional requirement for histological confirmation. It has long been recognized that technetium-labeled bone scintigraphy tracers can localize to myocardial amyloid deposits, and use of this imaging modality for the diagnosis of cardiac ATTR amyloidosis has lately been revisited. We conducted a multicenter study to ascertain the diagnostic value of bone scintigraphy in this disease.
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              Diagnosis and treatment of cardiac amyloidosis: a position statement of the ESC Working Group on Myocardial and Pericardial Diseases

              Cardiac amyloidosis is a serious and progressive infiltrative disease that is caused by the deposition of amyloid fibrils at the cardiac level. It can be due to rare genetic variants in the hereditary forms or as a consequence of acquired conditions. Thanks to advances in imaging techniques and the possibility of achieving a non-invasive diagnosis, we now know that cardiac amyloidosis is a more frequent disease than traditionally considered. In this position paper the Working Group on Myocardial and Pericardial Disease proposes an invasive and non-invasive definition of cardiac amyloidosis, addresses clinical scenarios and situations to suspect the condition and proposes a diagnostic algorithm to aid diagnosis. Furthermore, we also review how to monitor and treat cardiac amyloidosis, in an attempt to bridge the gap between the latest advances in the field and clinical practice.
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                Author and article information

                Contributors
                Role: Academic Editor
                Journal
                Diagnostics (Basel)
                Diagnostics (Basel)
                diagnostics
                Diagnostics
                MDPI
                2075-4418
                30 May 2021
                June 2021
                : 11
                : 6
                : 996
                Affiliations
                [1 ]Department of Medicine, Jacobi Medical Center, Albert Einstein College of Medicine, 1400 Pelham Parkway South, Bronx, NY 10461, USA; uppald@ 123456nychhc.org (D.U.); wangy19@ 123456nychhc.org (Y.C.W.); xux7@ 123456nychhc.org (X.X.)
                [2 ]Section of Cardiovascular Medicine, Yale University School of Medicine, 333 Cedar Street, New Haven, CT 06510, USA; damianos.kokkinidis@ 123456yale.edu
                [3 ]Department of Radiology, Division of Nuclear Medicine, Montefiore Medical Center, Albert Einstein College of Medicine, 111 East 210th Street, Bronx, NY 10467, USA; mtravin@ 123456montefiore.org
                [4 ]Department of Medicine, Division of Cardiology, Montefiore Medical Center, Albert Einstein College of Medicine, 111 East 210th Street, Bronx, NY 10467, USA; jtauras@ 123456montefiore.org
                Author notes
                [* ]Correspondence: liw11@ 123456nychhc.org
                Author information
                https://orcid.org/0000-0003-2821-5022
                Article
                diagnostics-11-00996
                10.3390/diagnostics11060996
                8228334
                34070853
                6337e983-6e03-42bb-8f2d-b47869dfcf81
                © 2021 by the authors.

                Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license ( https://creativecommons.org/licenses/by/4.0/).

                History
                : 16 May 2021
                : 27 May 2021
                Categories
                Review

                cardiac amyloidosis,cardiac scintigraphy,positron emission tomography

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