Key result
Late-onset ARVC confirmed by pathogenic PKP2 mutation can mimic ACS.
Why the study?
Arrhythmogenic right ventricular cardiomyopathy typically presents in younger individuals, making late-onset presentations mimicking acute coronary syndrome rare.
Case Report (n=1)
ARVC should be considered in the differential diagnosis of elderly patients presenting with unexplained ventricular arrhythmias and chest pain, particularly when coronary angiographic findings are normal.
Hypothesis-generating for late-onset ARVC mimicking ACS in elderly; prospective studies needed before diagnostic changes.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a cardiac condition characterized by the replacement of myocardial tissue with fibrofatty tissue, primarily affecting the right ventricle. Though typically presenting in younger individuals, this case report discusses a rare presentation in a 72-year-old female who exhibited symptoms mimicking acute coronary syndrome (ACS), including chest pain and sustained ventricular tachycardia (VT). Coronary angiography revealed normal coronary anatomy, ruling out ACS as the underlying cause. Cardiac MRI demonstrated structural abnormalities in the right ventricle, consistent with ARVC. Genetic testing confirmed a pathogenic mutation in the PKP2 gene. The patient was treated with antiarrhythmic medications and received an implantable cardioverter-defibrillator (ICD) for secondary prevention of sudden cardiac death. This report emphasizes the importance of considering ARVC in the differential diagnosis of elderly patients with unexplained ventricular arrhythmias and chest pain, particularly when coronary angiographic findings are normal.
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Hein et al. (2025) conducted a case report in Arrhythmogenic right ventricular cardiomyopathy (n=1). Implantable cardioverter-defibrillator (ICD) and sotalol was evaluated. A 72-year-old female presenting with symptoms mimicking acute coronary syndrome was diagnosed with late-onset arrhythmogenic right ventricular cardiomyopathy confirmed by a pathogenic PKP2 mutation.
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