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Review
. 2022 Apr 5;79(13):1288-1303.
doi: 10.1016/j.jacc.2022.01.036.

Critical Comparison of Documents From Scientific Societies on Cardiac Amyloidosis: JACC State-of-the-Art Review

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Free article
Review

Critical Comparison of Documents From Scientific Societies on Cardiac Amyloidosis: JACC State-of-the-Art Review

Claudio Rapezzi et al. J Am Coll Cardiol. .
Free article

Abstract

Over the last year, 5 national or international scientific societies have issued documents regarding cardiac amyloidosis (CA) to highlight the emerging clinical science, raise awareness, and facilitate diagnosis and management of CA. These documents provide useful guidance for clinicians managing patients with CA, and all include: 1) an algorithm to establish a diagnosis; 2) an emphasis on noninvasive diagnosis with the combined use of bone scintigraphy and the exclusion of a monoclonal protein; and 3) indications for novel disease-modifying therapies for symptomatic CA, either with or without peripheral neuropathy. Nonetheless, the documents diverge on specific details of diagnosis, risk stratification, and treatment. Highlighting the similarities and differences of the documents by the 5 scientific societies with respect to diagnosis, risk stratification, and treatment offers useful insight into the knowledge gaps and unmet needs in the management of CA. An analysis of these documents, therefore, highlights "gray zones" requiring further investigation.

Keywords: cardiac amyloidosis; diagnosis; guidelines; management; scientific societies.

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Conflict of interest statement

Funding Support and Author Disclosures Prof Rapezzi has served as a speaker and has received consulting fees from Pfizer, Alnylam, and Eidos; and has received research grant support to his institution from Pfizer. Prof Emdin, Drs Vergaro and Aimo has received consulting income from Pfizer; and their institution has received clinical trial funding from Pfizer. Dr Sinagra has served as a consultant for Novartis, AstraZeneca, Dompè, Impulse Dynamics, and Biotronik; and has received fees at congresses from Novartis, Bayer, AstraZeneca, Boston Scientific, Vifor Pharma, Menarini, and Akcea Therapeutics. Dr Merlo has received fees at congresses for Pfizer and Vifor Pharma; and has received research grant support from Pfizer. Dr Fontana has served as a consultant for Pfizer, Intellia, Alnylam, Ionis, Janssen, Novo Nordisk, and Akcea; and has received research grants from Pfizer and Eidos. Dr Maurer has received grant support from NIH R01HL139671, R21AG058348, and K24AG036778; has received consulting income from Intellia, Novo-Nordisk, Pfizer, Eidos, Prothena, Akcea, and Alnylam; and his institution has received clinical trial funding from Pfizer, Prothena, Eidos, and Alnylam. Dr Garcia-Pavia has served as a speaker and received consulting fees from Pfizer, Alnylam, Akcea, Neuroimmune, and Eidos; and has received research grant support to his institution from Pfizer. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

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