Current and Evolving Multimodality Cardiac Imaging in Managing Transthyretin Amyloid Cardiomyopathy.

Amyloid transthyretin (ATTR) amyloidosis is a protein-misfolding disease characterized by fibril accumulation in the extracellular space that can result in local tissue disruption and organ dysfunction. Cardiac involvement drives morbidity and mortality, and the heart is the major organ affected by ATTR amyloidosis. Multimodality cardiac imaging (ie, echocardiography, scintigraphy, and cardiac magnetic resonance) allows accurate diagnosis of ATTR cardiomyopathy (ATTR-CM), and this is of particular importance because ATTR-targeting therapies have become available and probably exert their greatest benefit at earlier disease stages. Apart from establishing the diagnosis, multimodality cardiac imaging may help to better understand pathogenesis, predict prognosis, and monitor treatment response. The aim of this review is to give an update on contemporary and evolving cardiac imaging methods and their role in diagnosing and managing ATTR-CM. Further, an outlook is presented on how artificial intelligence in cardiac imaging may improve future clinical decision making and patient management in the setting of ATTR-CM.

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Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

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Funding Support and Author Disclosures This work was supported by the GAMBIT foundation. The Bern University Hospital (Inselspital Bern) has received grants from Pfizer for the SWISS-CARE Amyloidosis registry. Dr Benz has received career development grants from the Swiss National Science Foundation; and has received reimbursement of travel expenses by Philips Healthcare and Amgen. Dr Cuddy has received research funding from Pfizer; has received honoraria for lectures from Ionis, BridgeBio, and Pfizer; and has received support for travel to meetings from Ionis. Dr Caobelli has received academic grant support from Mallinckrodt AG and Tillots AG; and has received speaker honoraria from Siemens Healthineers and Bracco. Dr Bernhard has received career development grants from the Swiss National Science Foundation. Dr Stämpfli has received consulting and speaker fees from Alnylam, Amgen, AstraZeneca, Bayer, Bristol-Myers Squibb, Pfizer, and Takeda. Dr Kwong has received research support from National Institutes of Health awards 1UH2 TR000901, 1RO1DK083424-01, and 1U01HL117006, Alnylam Pharmaceuticals, and the Society for Cardiovascular Magnetic Resonance. Dr Falk has received research funding from GlaxoSmithKline and Akcea; and has received consulting fees from Ionis, Alnylam Pharmaceuticals, and Caelum Biosciences. Dr Dorbala has received institutional grants from Pfizer, Attralus, GE Healthcare, Philips, the National Institutes of Health, and the American Heart Association; and has received payment for lectures from Janssen and Ionetix. Dr Gräni has received funding support from the Swiss National Science Foundation, InnoSuisse, the CAIM foundation, the GAMBIT foundation, and the Novartis Biomedical Research Foundation. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.