Myocarditis and ARVC/D: Variants or mimics?
Section snippets
Overview
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a disease characterized by fibrofatty replacement of the right ventricular (RV) myocardium, which is generally considered to be based on variations in desmosomal genes. However, its pathogenesis is not completely clear since myocarditis and high-intensity athletics have both been postulated to contribute to the onset and progression of the disease pattern. We observed a 21-year-old woman who presented with fulminant
Case report
A 21-year-old previously healthy Asian-American woman presented with a 3-day history of chest pain and gastrointestinal symptoms. Her initial assessment revealed hypotensive shock and accelerated ventricular rhythm with AV dissociation and prominent ST-segment elevation in leads V1–V3 (Figure 1). Laboratory investigation showed elevated troponin (12.79 ng/mL, normal <0.03 ng/mL). No coronary artery disease was found using coronary angiography. Initial echocardiogram with only inotropic support
Discussion
ARVC/D is a significant cause of ventricular arrhythmias and sudden cardiac death in young adults, including athletes. Since the recognition of a familial pattern and the subsequent discovery of pathogenic mutations in the desmosomal genes in families with ARVC/D, the disruption of the desmosomal structure is widely accepted as the main component of disease development,4 even though both desmosomal and nondesmosomal genes have been associated with ARVC/D.4 Nonetheless, the utility of genetic
Conclusion
We present a case of young woman with fulminant lymphocytic myocarditis who subseqently fulfilled the diagnosis criteria for ARVC/D. This report supports the hypothesis of a common pathophysiology of ARVC/D associated with desmosomal dysfunction, which can be based on inherited susceptibility, acquired disease injury, and/or extreme physiological volume overload and stretching. However, this case demonstrates the ambiguities in differentiating these disease processes and highlights the
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