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Die essentielle Myokardiopathie

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Zusammenfassung

  1. 1.

    Es gibt eine Herzhypertrophie unbekannter Ursache; auch die Pathogenese dieser Hypertrophie ist unklar. Die Krankheit wird zu den Myokardiopathien gezählt.

  2. 2.

    Es wurde eine Essentielle Myokardiopathie gefunden, die durch eine langsam fortschreitende Herzinsuffizienz bei zumeist jungen Männern mit großen Herzen und mit den Zeichen einer unerklärlichen Linkshypertrophie im Ekg gekennzeichnet ist.

  3. 3.

    Das klinische Syndrom dieser Essentiellen Myokardiopathie wurde bei 4 Kranken beobachtet, deren Autopsie eine starke Hypertrophie vor allem der Muskulatur der linken Herzkammer bei gleichzeitiger partieller oder diffuser Fibrose ergab. Ein 5. Kranker, dessen Herz das gleiche histologische Bild zeigte, war nach einem “stenokardischen” Herzanfall gestorben.

  4. 4.

    Bei 12 weiteren Kranken wurde ein ähnliches klinisches Bild gesehen. Alle Kranken mit Essentieller Myokardiopathie waren Männer.

  5. 5.

    Die Essentielle Myokardiopathie wird durch das Syndrom einer unerklärlichen Linkshypertrophie bei langsam größer werdendem Herzen und durch den Ausschluß aller möglichen Ursachen eines Herzfehlers oder einer Herzerkrankung wahrscheinlich gemacht.

  6. 6.

    Die Krankheit beginnt schleichend und verläuft langsam; aber sie schreitet über kurze oder längere Zeiten der Besserung unaufhaltsam fort. Die Behandlung kann nur eine symptomatische sein.

Summary

  1. 1.

    There are patients with cardiac hypertrophy of unknown etiology; the pathogenesis of this hypertrophy is not evident. This disease belongs to the “Myocardiopathies”.

  2. 2.

    There is described in this paper a distinct syndrome called “Essentielle Myokardiopathie” having a definite clinical pattern; this syndrome is characterized by a moderate progressive congestive heart failure; this condition was found particularly in young male adults having considerable cadiac enlargement and unexplained left ventricular hypertrophy in the electrocardiographic tracing.

  3. 3.

    This clinical syndrome of “Essentielle Myokardiopathie” was seen in 4 subjects, the autopsies of which revealed a striking hypertrophy of the myocardium particularly of the left ventricle with varying degrees of mural fibrosis. The fifth patient whose heart showed the same histological picture died after an attack of “substernal discomfort”.

  4. 4.

    Another 12 patients showed similar clinicald features. All subjects taken with “Essentielle Myokardiopathie” were male.

  5. 5.

    The “Essentielle Myokardiopathie” is suggested by the syndrome of an unexplained left ventricular hypertrophy and progressive cardiac enlargement, if all possible causes of valvular or other heart disease are excluded.

  6. 6.

    This disease characterized by light symptoms at the start runs a slow course. Inspite of short or long periods of improvement the process is progressive. Symptomatic treatment only is possible.

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  1. E. J. Haupt &  J. Schmidt

    Present address: Medizinischen Poliklinik der Universität Erlangen-Nürnberg, Östliche Stadtmauerstr. 29, 852, Erlangen

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    Herrn Prof. Dr.C. Korth zum 65. Geburtstag gewidmet.

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    Haupt, E.J., Schmidt, J. Die essentielle Myokardiopathie. Archiv für Kreislaufforschung 55, 4–26 (1968). https://doi.org/10.1007/BF02119692

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