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De Gaspari et al. Vessel Plus 2022;6:57 Vessel Plus
DOI: 10.20517/2574-1209.2022.05

Review Open Access

Cardiac amyloidosis: the pathologist’s point of view

1
1,2
Monica De Gaspari , Nicoletta Finato , Cristina Basso 2
1
Department of Medical Area (DAME), University of Udine, Udine 61-35121, Italy.
2
Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua - Azienda Ospedaliera, Padova
61-35121, Italy.
Correspondence to: Dr. Monica De Gaspari. Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of
Padua - Azienda Ospedaliera, Padova. Via Gabelli, Padua 61-35121, Italy. E-mail: monica.degaspari@phd.unipd.it

How to cite this article: De Gaspari M, Finato N, Basso C. Cardiac amyloidosis: the pathologist’s point of view. Vessel Plus
2022;6:57. https://dx.doi.org/10.20517/2574-1209.2022.05

Received: 20 Jan 2022 First Decision: 6 Apr 2022 Revised: 23 Apr 2022 Accepted: 10 May 2022 Published: 17 Oct 2022
Academic Editor: Alexander D. Verin Copy Editor: Haixia Wang Production Editor: Haixia Wang

Abstract
Cardiac amyloidosis is a well-known entity recently recognized as a common etiology of heart failure. This
infiltrative disease is caused by the deposition of misfolded proteins within the heart. The most common types of
cardiac amyloidosis result from fibrils composed of monoclonal immunoglobulin light chains or transthyretin.
Clinical presentation is usually elusive, and this can result in diagnostic delay. Diagnosis can be reached with non-
invasive methods, but it often requires tissue sampling with pathological analysis. It is fundamental to determine
the type of protein being deposited in order to indicate the specific treatment. In this article, we review the main
features of cardiac amyloidosis with a focus on different pathological presentations of this rare disorder.
Keywords: Cardiac amyloidosis, cardiovascular pathology, histology

INTRODUCTION
Amyloidosis is a disorder caused by abnormal conformation and metabolism of proteins resulting in
extracellular deposition of fibrillar material. These aggregates can alter tissue architecture and subsequently
affect organ function throughout the body. The disease is usually classified according to the precursor
proteins that undergo the misfolding and accumulation: more than 30 different human proteins have been
recognized as precursors for the buildup of amyloid deposits. Nevertheless, only nine misfolded proteins
can accumulate in the myocardium and be responsible for cardiac amyloidosis (CA) . Once considered a
[1]
rare disease, CA is now acknowledged as an underrecognized cause of heart failure, with a variable

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