Diagnosis and treatment of cardiac amyloidosis: A position statement of the ESC Working Group on Myocardial and Pericardial Diseases

Pablo Garcia-Pavia*, Claudio Rapezzi, Yehuda Adler, Michael Arad, Cristina Basso, Antonio Brucato, Ivana Burazor, Alida L.P. Caforio, Thibaud Damy, Urs Eriksson, Marianna Fontana, Julian D. Gillmore, Esther Gonzalez-Lopez, Martha Grogan, Stephane Heymans, Massimo Imazio, Ingrid Kindermann, Arnt V. Kristen, Mathew S. Maurer, Giampaolo MerliniAntonis Pantazis, Sabine Pankuweit, Angelos G. Rigopoulos, Ales Linhart

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

618 Scopus citations

Abstract

Cardiac amyloidosis is a serious and progressive infiltrative disease that is caused by the deposition of amyloid fibrils at the cardiac level. It can be due to rare genetic variants in the hereditary forms or as a consequence of acquired conditions. Thanks to advances in imaging techniques and the possibility of achieving a non-invasive diagnosis, we now know that cardiac amyloidosis is a more frequent disease than traditionally considered. In this position paper the Working Group on Myocardial and Pericardial Disease proposes an invasive and non-invasive definition of cardiac amyloidosis, addresses clinical scenarios and situations to suspect the condition and proposes a diagnostic algorithm to aid diagnosis. Furthermore, we also review how to monitor and treat cardiac amyloidosis, in an attempt to bridge the gap between the latest advances in the field and clinical practice.

Original languageEnglish
Pages (from-to)1554-1568
Number of pages15
JournalEuropean Heart Journal
Volume42
Issue number16
DOIs
StatePublished - 21 Apr 2021

Funding

FundersFunder number
Alnylam
National Institutes of HealthR21AG058348, K24AG036778
National Heart, Lung, and Blood InstituteR01HL139671
Pfizer
GlaxoSmithKline
Sanofi
Alexion Pharmaceuticals
Sanofi Genzyme
British Heart FoundationPI18/0765, FS/18/21/33447
Instituto de Salud Carlos IIIPI20/01379

    Keywords

    • AL
    • Amyloidosis
    • Cardiac amyloidosis
    • Diagnosis
    • TTR
    • Transthyretin
    • Treatment

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