Journal Article Ahead of print
PubMed: 42244336 DOI: 10.1093/eurheartj/ehag297
Arrhythmogenic right ventricular cardiomyopathy.
Eur Heart J
Authors: Alessio Gasperetti, Cynthia A James, Firat Duru, Peter van Tintelen, Hugh Calkins
Affiliations
- Division of Cardiology, Department of Medicine, Johns Hopkins University, 600 N. Wolfe St. Blalock 545, Baltimore, MD 21784, USA.
- Division of Cardiology, University Hospital of Zurich, Zurich, Switzerland.
- Department of Genetics, University Medical Center Utrecht, Utrecht, The Netherlands.
Abstract
Arrhythmogenic cardiomyopathy (ACM) is a form of inheritable cardiomyopathy characterized by ventricular scarring and/or fibro-fatty replacement. ACM is associated with a significant risk of sudden cardiac death due to ventricular arrhythmias, especially in young patients and endurance athletes. Aim of this review is to summarize the changing in understanding and framing of this multifaceted disease, in particular highlighting the current transition from a phenotype-first to a genotype-first framework for diagnosis and management.
MeSH
Cardiology, Arrhythmogenic Right Ventricular Dysplasia, Cardiomyopathies