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Transthyretin amyloid cardiomyopathy: An uncharted territory awaiting discovery

Transthyretin amyloid cardiomyopathy (ATTR-AC) is an under-recognized and underdiagnosed disease. Although traditionally considered a rare condition, the epidemiology of the disease is rapidly changing due to the possibility of non-invasive diagnosis through cardiac scintigraphy with bone tracers an...

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Autores principales: Porcari, Aldostefano, Merlo, Marco, Rapezzi, Claudio, Sinagra, Gianfranco
Formato: Online Artículo Texto
Lenguaje:English
Publicado: European Federation of Internal Medicine. Published by Elsevier B.V. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7534738/
https://www.ncbi.nlm.nih.gov/pubmed/33032855
http://dx.doi.org/10.1016/j.ejim.2020.09.025
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author Porcari, Aldostefano
Merlo, Marco
Rapezzi, Claudio
Sinagra, Gianfranco
author_facet Porcari, Aldostefano
Merlo, Marco
Rapezzi, Claudio
Sinagra, Gianfranco
author_sort Porcari, Aldostefano
collection PubMed
description Transthyretin amyloid cardiomyopathy (ATTR-AC) is an under-recognized and underdiagnosed disease. Although traditionally considered a rare condition, the epidemiology of the disease is rapidly changing due to the possibility of non-invasive diagnosis through cardiac scintigraphy with bone tracers and novel disease-modifying treatments providing survival advantages. Nevertheless, many questions and grey areas have to be addressed, such as the natural history of ATTR-AC, the role and implications of genotype–phenotype interactions, the best clinical management, prognostic stratification and the most appropriate treatments, including those already recommended for patients with heart failure. Clinicians have to cope with old beliefs and evolving concepts in ATTR-AC. A wide horizon of possibilities for physicians of many specialties is unfolding and awaits discovery.
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spelling pubmed-75347382020-10-06 Transthyretin amyloid cardiomyopathy: An uncharted territory awaiting discovery Porcari, Aldostefano Merlo, Marco Rapezzi, Claudio Sinagra, Gianfranco Eur J Intern Med Review Article Transthyretin amyloid cardiomyopathy (ATTR-AC) is an under-recognized and underdiagnosed disease. Although traditionally considered a rare condition, the epidemiology of the disease is rapidly changing due to the possibility of non-invasive diagnosis through cardiac scintigraphy with bone tracers and novel disease-modifying treatments providing survival advantages. Nevertheless, many questions and grey areas have to be addressed, such as the natural history of ATTR-AC, the role and implications of genotype–phenotype interactions, the best clinical management, prognostic stratification and the most appropriate treatments, including those already recommended for patients with heart failure. Clinicians have to cope with old beliefs and evolving concepts in ATTR-AC. A wide horizon of possibilities for physicians of many specialties is unfolding and awaits discovery. European Federation of Internal Medicine. Published by Elsevier B.V. 2020-12 2020-10-05 /pmc/articles/PMC7534738/ /pubmed/33032855 http://dx.doi.org/10.1016/j.ejim.2020.09.025 Text en © 2020 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved. Since January 2020 Elsevier has created a COVID-19 resource centre with free information in English and Mandarin on the novel coronavirus COVID-19. The COVID-19 resource centre is hosted on Elsevier Connect, the company's public news and information website. Elsevier hereby grants permission to make all its COVID-19-related research that is available on the COVID-19 resource centre - including this research content - immediately available in PubMed Central and other publicly funded repositories, such as the WHO COVID database with rights for unrestricted research re-use and analyses in any form or by any means with acknowledgement of the original source. These permissions are granted for free by Elsevier for as long as the COVID-19 resource centre remains active.
spellingShingle Review Article
Porcari, Aldostefano
Merlo, Marco
Rapezzi, Claudio
Sinagra, Gianfranco
Transthyretin amyloid cardiomyopathy: An uncharted territory awaiting discovery
title Transthyretin amyloid cardiomyopathy: An uncharted territory awaiting discovery
title_full Transthyretin amyloid cardiomyopathy: An uncharted territory awaiting discovery
title_fullStr Transthyretin amyloid cardiomyopathy: An uncharted territory awaiting discovery
title_full_unstemmed Transthyretin amyloid cardiomyopathy: An uncharted territory awaiting discovery
title_short Transthyretin amyloid cardiomyopathy: An uncharted territory awaiting discovery
title_sort transthyretin amyloid cardiomyopathy: an uncharted territory awaiting discovery
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7534738/
https://www.ncbi.nlm.nih.gov/pubmed/33032855
http://dx.doi.org/10.1016/j.ejim.2020.09.025
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