Arq. Bras. Cardiol. 2024; 121(7): e20230815
Unveiling Arrhythmogenic Right Ventricular Cardiomyopathy in Scleroderma
Introduction
Systemic sclerosis (SSc) patients commonly report exertional dyspnea and pulmonary hypertension should be first suspected, as the prevalence in these patients is reported to be 5-19% and it is associated with worse clinical outcomes. Additional differential diagnosis includes congenital left-to-right shunt and arrhythmogenic right ventricular cardiomyopathy (ARVC) when right ventricular dilatation and systolic dysfunction are present. Our case concerns an elderly lady with SSc who was initially diagnosed with precapillary pulmonary hypertension.
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