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Review
. 2025 Apr;18(4):e011796.
doi: 10.1161/CIRCHEARTFAILURE.124.011796. Epub 2025 Feb 18.

Guideline-Directed Medical Therapy for Heart Failure in Transthyretin Amyloid Cardiomyopathy

Stéphanie Kristina Schwarting  1   2 , Thomas Bieber  3   4 , Daniel R Davies  5 , Fabian Aus dem Siepen  2 , Julian Schwarting  6 , Ulrich Grabmaier  1   7 , Steffen Massberg  1   7 , Mathew S Maurer  8 , Stefan Kääb  1   7   9
Affiliations
Review

Guideline-Directed Medical Therapy for Heart Failure in Transthyretin Amyloid Cardiomyopathy

Stéphanie Kristina Schwarting et al. Circ Heart Fail. 2025 Apr.

Abstract

Cardiac amyloidosis is an underdiagnosed cause of infiltrative cardiomyopathy, leading to heart failure across the spectrum of ejection fractions. Although there are approved disease-modulating therapies for the transthyretin subtype (transthyretin amyloid cardiomyopathy [ATTR-CM]), the role of heart failure medications remains uncertain and challenging in clinical practice. Their effects on clinical outcomes, such as mortality and hospitalization, are unknown for ATTR-CM. This review aims to explore the use of these medications in ATTR-CM, considering the disease's stage and patient-specific issues, such as fluid homeostasis, autonomic dysfunction, conduction disorders, low and fixed stroke volumes, and decreased functional capacity. As our understanding of this condition deepens, it is important to reassess the impact of contemporary heart failure medication in ATTR-CM. Finally, the relevance of guideline recommendations for heart failure drugs based on left ventricular ejection fraction should be reconsidered in the context of ATTR-CM.

Keywords: amyloidosis; cardiomyopathies; heart failure; hospitalization.

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Conflict of interest statement

Dr S.K. Schwarting received speaker honoraria from and serves as a consultant for Pfizer, and travel expenses were disbursed by Alnylam Pharmaceuticals. Dr Bieber has been a speaker and a consultant for AbbVie, Affibody, Almirall, AnaptysBio, Arena, Asana Biosciences, ASLAN pharma, Bayer Health, BioVerSys, Boehringer Ingelheim, Bristol Myers Squibb, Connect Pharma, Dermavant, DIECE Therapeutics, Domain Therapeutics, EQRx, Galderma, Genzyme, Glenmark, GSK, Incyte, Innovaderm, IQVIA, Janssen, Kirin, Kymab, LEO, LG Chem, Lilly, L´Oréal, MSD, Novartis, Numab, OM-Pharma, Pfizer, Pierre Fabre, Q32bio, RAPT, Sanofi/Regeneron, and UCB. He is the founder and chairman of the board of the nonprofit biotech Davos Biosciences. Dr aus dem Siepen reports speaker fees from Pfizer, Ionis Pharmaceuticals, Alnylam Pharmaceuticals, and Janssen and consulting fees from Pfizer and Akcea Therapeutics. Dr Grabmaier receives speaker honoraria from AstraZeneca and Abbott. Dr Maurer reports grant support from the National Institutes of Health (R01HL139671 and R01AG081582-01); grants and personal fees from Alnylam, Pfizer, Eidos, Prothena, and Ionis; and personal fees from AstraZeneca, Akcea, Intellia, and Novo Nordisk. Dr Kääb received speaker honoraria from Pfizer and Bristol Myers Squibb. The other authors report no conflicts.

MeSH terms

Supplementary concepts