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Right Ventricular Dysplasia: A Report of 24 Adult Cases

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Myocarditis Cardiomyopathy

Part of the book series: International Boehringer Mannheim Symposia ((BOEHRINGER))

Abstract

Right ventricular dysplasia (RVD) is a pathological condition primarily affecting the right ventricle. The right ventricular musculature is partially or totally absent and is replaced by fatty and fibrous tissue. If the dysplasia is extensive, gross cardiomegaly will be evident on X-ray and the presenting clinical symptom may be congestive heart failure in infancy or early childhood. Under these circumstances the right ventricle is paper thin and there is apposition of the endocardium to the epicardium. This condition has been given the eponym, Uhl’s anomaly [2]. The patient who has almost complete absence of the myocardium of the right ventricle may survive to adulthood and may have asymptomatic cardiomegaly or recurrent ventricular arrhythmias. When the dysplasia is limited to portions of the right ventricle, cardiac dysfunction may be only minimally impaired. The patient may present in childhood or as an adult with cardiomegaly, ventricular premature beats, or ventricular tachycardia of right ventricular origin. The form of RVD with ventricular tachycardia as the principal manifestation has been termed “arrhythmogenic” [3–7]. It is the purpose of this report to describe our experience with the diagnosis and treatment of RVD in 24 adult patients referred to La Salpetriere Hospital, Paris, or to the Jean-Rostand Hospital, Paris-Ivry from 1973 to 1980. A detailed description of these cases has been published [4].

Financed in part by a grant from the Flinn Foundation, Phoenix, Arizona in support of Dr. Marcus’ sabbatical

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Authors
  1. F. I. Marcus
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  2. G. H. Fontaine
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  3. G. Guiraudon
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  4. R. Frank
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  5. J. L. Laurenceau
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  6. C. Malergue
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  7. Y. Grosgogeat
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Editor information

Editors and Affiliations

  1. Abteilung Innere Medizin III Kardiologie, Med. Universitätsklinik, Hugstetter Straße 55, D-7800, Freiburg i. Br., Federal Republic of Germany

    Hansjörg Just

  2. II. Med. Klinik u. Poliklinik, Universität Mainz, Langenbeckstraße 1, D-6500, Mainz, Federal Republik of Germany

    Hans Peter Schuster

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© 1983 Springer-Verlag Berlin Heidelberg

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Marcus, F.I. et al. (1983). Right Ventricular Dysplasia: A Report of 24 Adult Cases. In: Just, H., Schuster, H.P. (eds) Myocarditis Cardiomyopathy. International Boehringer Mannheim Symposia. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-68608-5_7

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  • DOI: https://doi.org/10.1007/978-3-642-68608-5_7

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-11617-2

  • Online ISBN: 978-3-642-68608-5

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