Prevention of Sudden Death and Management of Ventricular Arrhythmias in Arrhythmogenic Cardiomyopathy

Alessandro Trancuccio; Deni Kukavica; Andrea Sugamiele; Andrea Mazzanti; Silvia G Priori

doi:10.1016/j.ccep.2023.04.004

Prevention of Sudden Death and Management of Ventricular Arrhythmias in Arrhythmogenic Cardiomyopathy

Card Electrophysiol Clin. 2023 Sep;15(3):349-365. doi: 10.1016/j.ccep.2023.04.004. Epub 2023 Jun 9.

Authors

Alessandro Trancuccio¹, Deni Kukavica¹, Andrea Sugamiele², Andrea Mazzanti¹, Silvia G Priori³

Affiliations

¹ Department of Molecular Medicine, University of Pavia, Pavia, Italy; Molecular Cardiology, IRCCS Istituti Clinici Scientifici Maugeri, Pavia, Italy.
² Department of Molecular Medicine, University of Pavia, Pavia, Italy.
³ Department of Molecular Medicine, University of Pavia, Pavia, Italy; Molecular Cardiology, IRCCS Istituti Clinici Scientifici Maugeri, Pavia, Italy. Electronic address: silvia.priori@icsmaugeri.it.

PMID: 37558305
DOI: 10.1016/j.ccep.2023.04.004

Abstract

Arrhythmogenic cardiomyopathy is an umbrella term for a group of inherited diseases of the cardiac muscle characterized by progressive fibro-fatty replacement of the myocardium. As suggested by the name, the disease confers electrical instability to the heart and increases the risk of the development of life-threatening arrhythmias, representing one of the leading causes of sudden cardiac death (SCD), especially in young athletes. In this review, the authors review the current knowledge of the disease, highlighting the state-of-the-art approaches to the prevention of the occurrence of SCD.

Keywords: Arrhythmogenic cardiomyopathy; Heart failure; Implantable cardioverter defibrillator; Sudden cardiac death; Ventricular arrhythmias.

Publication types

Review
Research Support, Non-U.S. Gov't

MeSH terms

Arrhythmias, Cardiac / etiology
Arrhythmogenic Right Ventricular Dysplasia* / complications
Death, Sudden, Cardiac / etiology
Defibrillators, Implantable* / adverse effects
Humans
Myocardium