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Sinagra et al. Vessel Plus 2022;6:44 Vessel Plus
DOI: 10.20517/2574-1209.2021.143

Editorial Open Access

The changing perspective on cardiac amyloidosis in

the modern era

Gianfranco Sinagra, Aldostefano Porcari
Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-
Isontina (ASUGI) and University of Trieste - Trieste, Trieste 34149, Italy.

Correspondence to: Prof. Gianfranco Sinagra, 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: gianfranco.sinagra@asugi.sanita.fvg.it
How to cite this article: Sinagra G, Porcari A. The changing perspective on cardiac amyloidosis in the modern era. Vessel Plus
2022;6:44. https://dx.doi.org/10.20517/2574-1209.2021.143
Received: 23 Nov 2021 Accepted: 7 Dec 2021 Published: 1 Aug 2022

Academic Editor: Wilbert Aronow Copy Editor: Haixia Wang Production Editor: Haixia Wang

INTRODUCTION
Recent years have witnessed a revolution in the traditional teachings and beliefs in cardiac amyloidosis
(CA). In a very short time, this condition has gone from a rare, underdiagnosed, and difficult-to-diagnose
disease with no specific treatment to being relatively prevalent, easy to diagnose, and treatable. This Special
Issue on cardiac amyloidosis in Vessel Plus was conceived with the contribution of internationally
recognized experts and opinion leaders in CA to provide updated, clinically relevant information for
cardiologists and physicians of different specialties who are involved in the care of patients with amyloidosis
as well as to discuss the many grey areas under investigation.

Amyloidosis: a history of failing biological systems
CA results from the extracellular deposition of misfolded proteins, mostly immunoglobulin light chain (AL)
produced by an abnormal clonal proliferation of bone marrow plasma cells and transthyretin (TTR)
[1]
[2]
protein . Age-related failure of homoeostatic mechanisms in wild-type TTR (wtTTR), destabilizing
mutations in variant TTR (vTTR), or a hematological disorder in AL amyloidosis can prompt protein
fibrillation . Irrespective of the specific protein precursor, increased wall thickness, biatrial dilatation, and
[2]
poor diastolic filling due to noncompliant ventricles are the hallmarks of CA , as discussed by De Gaspari
[2,3]
[4]
et al. .

© The Author(s) 2022. Open Access This article is licensed under a Creative Commons Attribution 4.0
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adaptation, distribution and reproduction in any medium or format, for any purpose, even commercially, as
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