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Kardiomyopathien

Teil I: Klassifikation der Kardiomyopathien — dilatative Kardiomyopathie

Cardiomyopathies. Part I: classification — dilated cardiomyopathy

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Zusammenfassung

Kardiomyopathien stellen eine wesentliche Ursache für die Entwicklung einer Herzinsuffizienz und den plötzlichen Herztod dar. Nach WHO-Klassifikation werden primäre bzw. „spezifische“ Kardiomyopathien mit bekannter Ätiologie von den „idiopathischen“ Erkrankungen des Herzmuskels mit kardialer Dysfunktion unklarer Ätiologie abgegrenzt. Die Klassifizierung erfolgt somit überwiegend nach pathophysiologischen Gesichtspunkten. Das diagnostische Spektrum bei Kardiomyopathien umfasst das gesamte Arsenal nichtinvasiver und invasiver kardiologischer Untersuchungsmethoden. Die exakte Diagnosesicherung einzelner Krankheitsbilder erfordert zudem spezielle Untersuchungstechniken. So können durch immunhistologische und molekularbiologische Untersuchungen nach Endomyokardbiopsie virusinduzierte inflammatorische Kardiomyopathien diagnostiziert werden, die möglicherweise von einer kausalen immunmodulatorischen Therapie profitieren. Bei genetischen/familiären Formen der DCM wurden bereits zahlreiche Genmutationen identifiziert. Erstgradige Verwandte sollten auf frühe Stadien der Erkrankung gescreent werden. Hinsichtlich der Primärprävention des plötzlichen Herztods zeichnet sich zunehmend die Überlegenheit des implantierbaren Defibrillators gegenüber einer medikamentösen Therapie (z. B. Amiodaron) ab.

Abstract

Cardiomyopathies are common causes of heart failure and sudden cardiac death. According to the WHO classification, “specific” cardiomyopathies are differentiated from “idiopathic” cardiomyopathies. Thus, this classification is primarily based on pathophysiological characteristics. The diagnostic spectrum in cardiomyopathies comprises the entire spectrum of non-invasive and invasive cardiological examination techniques. The exact verification of certain cardiomyopathies necessitates additionally investigations. For example, immunohistological and molecular biological investigations of endomyocardial biopsies may confirm inflammatory cardiomyopathy, which is often induced by viruses. Several studies have shown that specific immunomodulatory treatment options can halt the progressive course of the disease. Several gene mutations have been identified in genetic/familial dilated cardiomyopathy. First-degree relatives should be screened for early stages. Primary prevention of sudden cardiac death shows increasing superiority of the implantable defibrillator compared with pharmacological approaches (i.e. amiodarone).

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Wir danken Frau Dr. Regina Gaub für die echokardiographischen Bilder.

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  1. Medizinische Klinik II, Kardiologie und Pneumologie, Charité — Universitätsmedizin Berlin

    H. P. Schultheiss, M. Noutsias, U. Kühl, W. Poller & M. Pauschinger

  2. Institut Kardiale Diagnostik und Therapie GmbH, Berlin

    D. Lassner & U. Gross

  3. Medizinische Klinik II, Charité — Campus Benjamin Franklin, Hindenburgdamm 30, 12200, Berlin

    H. P. Schultheiss

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  1. H. P. Schultheiss
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Schultheiss, H.P., Noutsias, M., Kühl, U. et al. Kardiomyopathien. Internist 46, 1245–1258 (2005). https://doi.org/10.1007/s00108-005-1483-9

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