Arrhythmogenic Right Ventricular Cardiomyopathy in a 20-year-old Woman with Systemic Sclerosis

Abstract

A 20-year-old Japanese woman with systemic sclerosis was evaluated for the progressive aggravation of chest pain, palpitations and dyspnea. Cardiac magnetic resonance imaging revealed diffuse wall thinning, segmental dyskinesis and late gadolinium enhancement of the right ventricular (RV) myocardium. Cardiac catheterization demonstrated no pulmonary hypertension. 24-hour electrocardiography monitoring showed non-sustained ventricular tachycardia (VT). Pulseless VT was induced via programmed ventricular stimulation. An endomyocardial biopsy of the right side of the interventricular septum was performed, the histological specimen of which demonstrated massive myocardial atrophy and fibro-fatty replacement with predominant fibrotic changes. The patient was ultimately diagnosed with arrhythmogenic RV cardiomyopathy.