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World Heart Federation Consensus on Transthyretin Amyloidosis Cardiomyopathy (ATTR-CM)

Brito, Dulce; Albrecht, Fabiano Castro; De Arenaza, Diego Perez; Bart, Nicole; Better, Nathan; Carvajal-Juarez, Isabel; Conceição, Isabel; ... Pinto, Fausto J; + view all (2023) World Heart Federation Consensus on Transthyretin Amyloidosis Cardiomyopathy (ATTR-CM). Global Heart , 18 (1) , Article 59. 10.5334/gh.1262. Green open access

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Abstract

Transthyretin amyloid cardiomyopathy (ATTR-CM) is a progressive and fatal condition that requires early diagnosis, management, and specific treatment. The availability of new disease-modifying therapies has made successful treatment a reality. Transthyretin amyloid cardiomyopathy can be either age-related (wild-type form) or caused by mutations in the TTR gene (genetic, hereditary forms). It is a systemic disease, and while the genetic forms may exhibit a variety of symptoms, a predominant cardiac phenotype is often present. This document aims to provide an overview of ATTR-CM amyloidosis focusing on cardiac involvement, which is the most critical factor for prognosis. It will discuss the available tools for early diagnosis and patient management, given that specific treatments are more effective in the early stages of the disease, and will highlight the importance of a multidisciplinary approach and of specialized amyloidosis centres. To accomplish these goals, the World Heart Federation assembled a panel of 18 expert clinicians specialized in TTR amyloidosis from 13 countries, along with a representative from the Amyloidosis Alliance, a patient advocacy group. This document is based on a review of published literature, expert opinions, registries data, patients’ perspectives, treatment options, and ongoing developments, as well as the progress made possible via the existence of centres of excellence. From the patients’ perspective, increasing disease awareness is crucial to achieving an early and accurate diagnosis. Patients also seek to receive care at specialized amyloidosis centres and be fully informed about their treatment and prognosis.

Type: Article
Title: World Heart Federation Consensus on Transthyretin Amyloidosis Cardiomyopathy (ATTR-CM)
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.5334/gh.1262
Publisher version: https://doi.org/10.5334/gh.1262
Language: English
Additional information: Copyright © 2023 The Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 International License (CC-BY 4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. See http://creativecommons.org/licenses/by/4.0/.
Keywords: Heart Failure; Amyloidosis; Transthyretin amyloid cardiomyopathy (ATTR-CM); diagnosis; treatment; patients’ perspective
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
URI: https://discovery.ucl.ac.uk/id/eprint/10180264
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