Key result
Cardiac MRI in a pediatric patient with desmoplakin cardiomyopathy revealed findings overlapping with myocarditis and left ventricular noncompaction.
Why the study?
There are no accepted clinical diagnostic criteria for arrhythmogenic left ventricular cardiomyopathy, and cardiac MRI can be instrumental in its diagnosis.
Case Report (n=1)
Cardiac MRI can identify structural and functional abnormalities in desmoplakin-related ALVC, which may overlap with myocarditis and LV noncompaction.
Answers are grounded in this paper and cite their sources.
CMR overlaps with myocarditis and LV noncompaction in pediatric desmoplakin cardiomyopathy; leaves diagnostic specificity open for larger studies.
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. Keywords: Pediatrics, Heart, Cardiomyopathies Supplemental material is available for this article.
No takes yet. Share an insight, caveat, or question.
Morcos et al. (2023) conducted a case report in Desmoplakin-related Arrhythmogenic Left Ventricular Cardiomyopathy (n=1). Cardiac MRI was evaluated. Cardiac MRI in a pediatric patient with desmoplakin cardiomyopathy revealed findings overlapping with myocarditis and left ventricular noncompaction.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: