Systemic Sclerosis Presenting as Arrythmogenic Cardiomyopathy: A Case Series

We present 2 cases of biventricular dysfunction and ventricular arrhythmia initially diagnosed as arrhythmogenic cardiomyopathy (ACM). However, given that both patients also had diagnoses of systemic sclerosis (SSc), and considering the rapid progression of biventricular dysfunction, the pattern of late gadolinium enhancement (LGE), endomyocardial biopsy (EMB) findings, and progressive extracardiac symptoms, the diagnosis was revised to SSc cardiomyopathy in both cases. This article highlights that SSc can present as cardiomyopathy meeting ACM criteria and provides valuable insights to help clinicians distinguish SSc heart disease from primary ACM.