IF 3 3区 医学Q2 CARDIAC & CARDIOVASCULAR SYSTEMSGlobal HeartPub Date : 2023-10-26eCollection Date: 2023-01-01DOI:10.5334/gh.1262
Dulce Brito, Fabiano Castro Albrecht, Diego Perez de Arenaza, Nicole Bart, Nathan Better, Isabel Carvajal-Juarez, Isabel Conceição, Thibaud Damy, Sharmila Dorbala, Jean-Christophe Fidalgo, Pablo Garcia-Pavia, Junbo Ge, Julian D Gillmore, Jacek Grzybowski, Laura Obici, Daniel Piñero, Claudio Rapezzi, Mitsuharu Ueda, Fausto J Pinto
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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. 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World Heart Federation Consensus on Transthyretin Amyloidosis Cardiomyopathy (ATTR-CM).
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.
Global HeartMedicine-Cardiology and Cardiovascular Medicine
CiteScore
5.70
自引率
5.40%
发文量
77
审稿时长
5 weeks
期刊介绍:
Global Heart offers a forum for dialogue and education on research, developments, trends, solutions and public health programs related to the prevention and control of cardiovascular diseases (CVDs) worldwide, with a special focus on low- and middle-income countries (LMICs). Manuscripts should address not only the extent or epidemiology of the problem, but also describe interventions to effectively control and prevent CVDs and the underlying factors. The emphasis should be on approaches applicable in settings with limited resources.
Economic evaluations of successful interventions are particularly welcome. We will also consider negative findings if important. While reports of hospital or clinic-based treatments are not excluded, particularly if they have broad implications for cost-effective disease control or prevention, we give priority to papers addressing community-based activities. We encourage submissions on cardiovascular surveillance and health policies, professional education, ethical issues and technological innovations related to prevention.
Global Heart is particularly interested in publishing data from updated national or regional demographic health surveys, World Health Organization or Global Burden of Disease data, large clinical disease databases or registries. Systematic reviews or meta-analyses on globally relevant topics are welcome. We will also consider clinical research that has special relevance to LMICs, e.g. using validated instruments to assess health-related quality-of-life in patients from LMICs, innovative diagnostic-therapeutic applications, real-world effectiveness clinical trials, research methods (innovative methodologic papers, with emphasis on low-cost research methods or novel application of methods in low resource settings), and papers pertaining to cardiovascular health promotion and policy (quantitative evaluation of health programs.