Arrhythmogenic right ventricular cardiomyopathy in a 20-year-old woman with systemic sclerosis.
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.
Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Japan.
Kaoru
K
Dohi
Shiro
S
Nakamori
Tairo
T
Kurita
Youichi
Y
Omoto
Masaki
M
Ishida
Mashio
M
Nakamura
Kyoko
K
Imanaka-Yoshida
Hajime
H
Sakuma
Hitoshi
H
Mizutani
Masaaki
M
Ito
D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D001706:Biopsy; D006328:Cardiac Catheterization; D003937:Diagnosis, Differential; D004562:Electrocardiography; D017809:Fatal Outcome; D005260:Female; D006352:Heart Ventricles / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods*; D012595:Scleroderma, Systemic / Q000150:complications* / Q000175:diagnosis; D016278:Ventricular Function, Right; D055815:Young Adult