MRI Findings in Desmoplakin-related Arrhythmogenic Left Ventricular Cardiomyopathy in a Pediatric Patient: A Case Report

Overview

Journal Radiol Cardiothorac Imaging

Specialty Cardiology & Vascular Diseases

Date 2023 May 1

PMID 37124635

Authors

George Morcos

Sudhir Vashist

Recai Aktay

Affiliations

Soon will be listed here.

Abstract

Arrhythmogenic cardiomyopathy (ACM) is a heart muscle disorder that cannot be explained by ischemic, hypertensive, or valvular heart disease and often results in sudden cardiac death. Arrhythmogenic right ventricular cardiomyopathy (ARVC) is the best-characterized ACM and can be diagnosed using the revised task force criteria. In contrast, there are no accepted clinical diagnostic criteria for arrhythmogenic left ventricular cardiomyopathy (ALVC), another subtype of ACM. Cardiac MRI aids in ARVC diagnosis by delineating biventricular structural and functional abnormalities and can be instrumental in diagnosing ALVC. This report presents a pediatric case of desmoplakin cardiomyopathy, a distinct subtype of ALVC, with findings overlapping myocarditis and LV noncompaction. Pediatrics, Heart, Cardiomyopathies Supplemental material is available for this article. © RSNA, 2023.

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