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

Brito, Dulce and Albrecht, Fabiano Castro and de Arenaza, Diego Perez and Bart, Nicole and Better, Nathan and Carvajal-Juarez, Isabel and Conceição, Isabel and Damy, Thibaud and Dorbala, Sharmila and Fidalgo, Jean-Christophe and Garcia-Pavia, Pablo and Ge, Junbo and Gillmore, Julian D. and Grzybowski, Jacek and Obici, Laura and Piñero, Daniel and Rapezzi, Claudio and Ueda, Mitsuharu and Pinto, Fausto J. (2023) World Heart Federation Consensus on Transthyretin Amyloidosis Cardiomyopathy (ATTR-CM). Global Heart, 18 (1). ISSN 2211-8179

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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.

Item Type: Article
Subjects: R Medicine > R Medicine (General)
Depositing User: Repository Administrator
Date Deposited: 01 May 2024 04:04
Last Modified: 01 May 2024 04:04

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