Arrhythmogenic cardiomyopathy: current updates and future challenges.
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Authors
Shah, Nihit
Issue Date
2024-07-04
Type
Article
Peer-Reviewed Publication
Peer-Reviewed Publication
Keywords
Arrhythmogenic cardiomyopathy
Cardiac magnetic resonance imaging
Desmosomal genes
Electrophysiology
Peer-reviewed article
Review
Cardiac magnetic resonance imaging
Desmosomal genes
Electrophysiology
Peer-reviewed article
Review
Journal
Review in Cardiovascular Medicine
Volume
25
Issue
6
Alternative Title
Abstract
Arrhythmogenic cardiomyopathy (ACM) epitomises a genetic anomaly hallmarked by a relentless fibro-fatty transmogrification of cardiac myocytes. Initially typified as a right ventricular-centric disease, contemporary observations elucidate a frequent occurrence of biventricular and left-dominant presentations. The diagnostic labyrinth of ACM emerges from its clinical and imaging properties, often indistinguishable from other cardiomyopathies. Precision in diagnosis, however, is paramount and unlocks the potential for early therapeutic interventions and vital cascade screening for at-risk individuals. Adherence to the criteria established by the 2010 task force remains the cornerstone of ACM diagnosis, demanding a multifaceted assessment incorporating electrophysiological, imaging, genetic, and histological data. Reflecting the evolution of our understanding, these criteria have undergone several revisions to encapsulate the expanding spectrum of ACM phenotypes. This review seeks to crystallise the genetic foundation of ACM, delineate its clinical and radiographic manifestations, and offer an analytical perspective on the current diagnostic criteria. By synthesising these elements, we aim to furnish practitioners with a strategic, evidence-based algorithm to accurately diagnose ACM, thereby optimising patient management and mitigating the intricate challenges of this multifaceted disorder.
Citation
Zathar Z, Shah N, Desai N, Patel PA. Arrhythmogenic Cardiomyopathy: Current Updates and Future Challenges. Rev Cardiovasc Med. 2024 Jun 4;25(6):208. doi: 10.31083/j.rcm2506208. PMID: 39076315; PMCID: PMC11270059.