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Japanese

Case reports of right ventricular dilated cardiomyopathy in 2 male siblings Naoto Endo 1,4 , Masaaki Kouda 1 , Makoto Kitamura 1 , Hirohisa Uwatoko 1 , Hiroaki Shiotu 1 , Masahiro Funauchi 1 , Akimasa Takasu 2 , Nobuo Kitamura 3 1Cardiovascular Division, Department of Internal Medicine, Kyoto City Hospital 2Division of Clinical Pathology, Kyoto City Hospital 3Division of Cardiovascular Surgery, Osaka National Hospital 4Present address;Matsushita Health Administration Center pp.99-104
Published Date 1988/1/15
DOI https://doi.org/10.11477/mf.1404205188
  • Abstract
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We report 2 male siblings (36 years old and 32 years old) who were recognized to be affected with right ventricular dilated cardiomyopathy. On the chest radiograms, cardio-thoratic ratio increased in the elder and was normal in the younger. The standard electrocardiograms indicated negative T waves in leads which reflect right ventricle in both cases. Holter's dynamic electrocardiograms in 24 hours showed no worthening arrhythmias except for occasional premature ventricular contractions in both cases. Two dimensional echocardiograms demon-strated more enlarged right ventricle and rather smallleft ventricle in these 2 cases, and this findings were more predominant in the elder. Both free right ventricular walls were denoted in diffuse hypokinetic movement with rather hypertrophic wall thickness, and dilated right atrium in the elder. The cardiac catheterization data of both cases were investigated as relative elevation of right atrial pressure with protruded "a" wave and also of right ventricular end-diastolic pressure, respectively, but these findings were predominant in the elder. Left ventricular end-diastolic pressures were in normal ranges. In the elder, cardic index decreased to 1.9l/min/m2, in the youner remained at 2.6l/min/m2. The contrast right ventriculogram of the elder indicated tricuspid regurgitation. In the histological studies of right ventricular endomyocardial biopsy specimens, myocardial cells illustrated irregular hypertrophies, disarraies, fatty infiltrations, and fibrous degenera-tions, respectively.

Hereditory factor was presumed in maternal line, that is, two male adults (an uncle and a cousin) died suddenly at their homes. But it was not clear that they have been suffered from some heart diseases.


Copyright © 1988, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1200 印刷版ISSN 0452-3458 医学書院

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