Key result
In a 28-year-old female with rapid progression of arrhythmogenic cardiomyopathy, histopathological examination following heart transplantation revealed concomitant myocarditis.
Why the study?
Arrhythmogenic cardiomyopathy predisposes patients to fatal ventricular arrhythmias and ventricular systolic dysfunction, and its relationship with myocarditis has gained significant attention.
Case Report (n=1)
Rapid progression of arrhythmogenic cardiomyopathy may indicate concomitant myocarditis, suggesting inflammation plays a role in the disease's natural history.
Answers are grounded in this paper and cite their sources.
Myocarditis may accelerate ACM progression; this case is hypothesis-generating for inflammatory modifiers in genetic cardiomyopathy.
BACKGROUND: Arrhythmogenic cardiomyopathy (ACM) is a genetic myocardial disease characterized by progressive myocyte loss and fibrofatty (fibrous and adipose) tissue replacement to predispose these patients to fatal ventricular arrhythmias and impairment of ventricular systolic function. The relationship of ACM and myocarditis has gained significant attention. CASE PRESENTATION: This case presented a 28-year-old female who was admitted to the hospital with complaints of recurrent lower limb edema and palpitations for 6 months. Her electrocardiogram revealed a typical manifestation of an advanced form of biventricular arrhythmogenic cardiomyopathy (ACM). Despite systematic medical management, her right ventricle (RV) function deteriorated rapidly, necessitating heart transplantation. Postoperative histopathological examinations confirmed the RV involvement as reflected in the electrocardiogram. Especially, multiple foci of lymphocytic infiltration were observed throughout the heart, with the RV being the most severe. CONCLUSION: When a rapid progression of ACM occurs, a concomitant myocarditis should be considered. ACM may be an inflammation-mediated transformation from myocardial tissue to fibrofatty tissue, and myocarditis may be a part of the natural history in some ACM cases.
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Li et al. (2025) conducted a case report in Arrhythmogenic cardiomyopathy and myocarditis (n=1). Concomitant myocarditis was evaluated on Histopathological diagnosis. In a 28-year-old female with rapid progression of arrhythmogenic cardiomyopathy, histopathological examination following heart transplantation revealed concomitant myocarditis.
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