IMR Press / RCM / Volume 23 / Issue 10 / DOI: 10.31083/j.rcm2310345
Open Access Review
Emerging from the Darkness. Sudden Cardiac Death in Cardiac Amyloidosis
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1 Fondazione Policlinico Universitario Campus-Biomedico, Operative Research Unit of Cardiovascular Science, 00128 Roma, Italy
2 Department of Cardiovascular Science, Università Campus Bio-Medico di Roma, 00128 Roma, Italy
3 Department of Haematology, Università Campus Bio-Medico di Roma, 00128 Rome, Italy
4 Laboratory of Medical Genetics, Università Campus Bio-Medico di Roma, 00128 Rome, Italy
*Correspondence: v.cammalleri@policlinicocampus.it (Valeria Cammalleri)
These authors contributed equally.
Academic Editor: John Lynn Jefferies
Rev. Cardiovasc. Med. 2022, 23(10), 345; https://doi.org/10.31083/j.rcm2310345
Submitted: 28 July 2022 | Revised: 12 September 2022 | Accepted: 13 September 2022 | Published: 14 October 2022
(This article belongs to the Special Issue Sudden Cardiac Death in Special Population—Volume 2)
Copyright: © 2022 The Author(s). Published by IMR Press.
This is an open access article under the CC BY 4.0 license.
Abstract

Cardiac amyloidosis (CA) manifests as infiltrative cardiomyopathy with a hypertrophic pattern, usually presenting with heart failure with a preserved ejection fraction. In addition, degenerative valvular heart disease, particularly severe aortic stenosis, is commonly seen in patients with CA. However, amyloid fibril deposition might also infiltrate the conduction system and promote the development of electrical disorders, including ventricular tachyarrhythmias, atrio-ventricular block or acute electromechanical dissociation. These manifestations can increase the risk of sudden cardiac death. This review summarises the pathophysiological mechanisms and risk factors for sudden cardiac death in CA and focuses on the major current concerns regarding medical and device management in this challenging scenario.

Keywords
implantable cardioverter defibrillator
sudden cardiac death
cardiac amyloidosis
monoclonal immunoglobulin light chains
transthyretin amyloidosis
ventricular arrhythmia
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