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Porcari et al. Vessel Plus 2022;6:33 Vessel Plus
DOI: 10.20517/2574-1209.2021.134

Review Open Access

Grey zones in the supportive treatments of cardiac

amyloidosis

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Aldostefano Porcari , Linda Pagura , Guerino Giuseppe Varrà , Maddalena Rossi , Francesca Longo ,
2,3
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Riccardo Saro , Davide Barbisan , Marco Cittar , Claudio Rapezzi , Marco Merlo 1
1
Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-
Isontina (ASUGI) and University of Trieste, Trieste 34149, Italy.
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Cardiothoracic Department, University of Ferrara, Ferrara 44100, Italy.
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Maria Cecilia Hospital, GVM Care & Research, Ravenna 48010, Italy.
Correspondence to: Prof. Marco Merlo. Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department,
Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI) and University of Trieste, Via Valdoni 1, Trieste 34149, Italy.
E-mail: marco.merlo79@gmail.com
How to cite this article: Porcari A, Pagura L, Varrà GG, Rossi M, Longo F, Saro R, Barbisan D, Cittar M, Rapezzi C, Merlo M. Grey
zones in the supportive treatments of cardiac amyloidosis. Vessel Plus 2022;6:33. https://dx.doi.org/10.20517/2574-
1209.2021.134
Received: 8 Nov 2021 First Decision: 8 Dec 2021 Revised: 15 Dec 2021 Accepted: 31 Dec 2021 Published: 1 Jun 2022
Academic Editor: Christopher Lau Copy Editor: Yue-Yue Zhang Production Editor: Yue-Yue Zhang
Abstract
Recent advances in the diagnosis and treatment of cardiac amyloidosis (CA) have translated into a longer life
expectancy of patients and more challenging clinical scenarios. Compared to the past, patients with CA and heart
failure (HF) currently encountered in clinical practice are a more heterogeneous population and require tailored
strategies. The perception of CA as a treatable disease has opened new possibilities for the management of these
patients, but many grey areas remain to be explored. The aim of this review is to provide practical suggestions for
daily clinical activity in the management of challenging scenarios in CA, including the effectiveness and tolerability
of evidence-based HF medication; rate vs. rhythm control in atrial fibrillation, thromboembolic risk, and
anticoagulation therapies; replacement of severe aortic valve stenosis; the impact of implantable cardioverter
defibrillator on survival; and the usefulness of cardiac resynchronization therapy.

Keywords: Cardiac amyloidosis, grey areas, clinical management, heart failure, atrial fibrillation, aortic stenosis,
arrhythmic risk stratification

© The Author(s) 2022. Open Access This article is licensed under a Creative Commons Attribution 4.0
International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, sharing,
adaptation, distribution and reproduction in any medium or format, for any purpose, even commercially, as
long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and
indicate if changes were made.

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