A Case of Arrhythmogenic Right Ventricular Cardiomyopathy followed up for 20 Yearswith referenc to the new classification of ARVC by Fontaine G et al. Tomooki Iwamoto 1 , Teruhisa Tanabe 1 , Mihoko Igarashi 1 , Minoru Aikawa 1 , Hiroyuki Sakai 1 , Motoyuki Yoshitake 1 , Shigeru Kusuzaki 1 , Shinya Goto 1 , Sumihisa Abe 1 , Shunnosuke Handa 1 1Department of Internal Medicine, School of Medicine, Tokai University Keyword: 不整脈源性右室心筋症 , 不整脈源性右室異形成症 , 心不全 , arrhythmogenic rigit ventricular cardiomyopathy , arrhythmogenic right ventricular dysplasia , heart failure pp.93-98
Published Date 1999/1/15
DOI https://doi.org/10.11477/mf.1404901836
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A 33-year-old woman with arrhythmogenic right ventricular cardiomyopathy followed up for 20 years was reported. She was found to have marked car- diomegaly in chest x-ray at age 13, though she did not have any manifestation of it until the age of 28, when she started to have dyspnea on exertion. At the age of 30, she developed symptoms of right ventricular failure and was admitted to the hospital repeatedly for its management. EKG recordings revealed multifocal ventricular arrhythmia with left bundle branch block pattern and epsilon waves which revealed a risk factor of severe ventricular arrhythmia. There was also late potential in signal-averaged EKG. The echocardiogram, ventriculography and magnetic resonance imaging showed right ventricular dilatation and thinning of the ventricular wall with poor left ventricular contraction. Myocardial biopsy showed hypertrophy and attenuation of myocells, interstitial infiltration of fat and fibrosis. Worsening of the right ventricular failure reached the NYHA class IV. We discussed the diagnostic problems in patients with ARVC, with reference to the new classification of ARVC by Fontaine G et al.

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