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Kardiale Sarkoidose

Cardiac sarcoidosis

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Der Kardiologe Aims and scope

Zusammenfassung

Die Sarkoidose ist eine systemisch granulomatöse Erkrankung ungeklärter Ätiologie, die histologisch durch nicht verkäsende Granulome charakterisiert ist. Zu den Prädilektionsorganen gehören Hiluslymphknoten, Lunge, Auge und Haut. Allerdings können auch andere Organe wie Herz, Leber, Milz, Knochen, Muskeln und nichtpulmonale Lymphknoten betroffen sein. Eine kardiale Beteiligung ist relativ selten, verschlechtert die Prognose jedoch deutlich. Die häufigsten klinischen Manifestationen einer kardialen Sarkoidose umfassen neben einer Herzinsuffizienz ventrikuläre Arrhythmien. Auch Fälle von einem plötzlichen Herztod sind bekannt. Das Fehlen einer klinischen Symptomatik schließt eine kardiale Manifestation der Sarkoidose nicht aus, da die meisten Fälle erst post mortem erkannt werden. Die Diagnose basiert auf einer Reihe von Untersuchungen wie Elektrokardiographie, Echokardiographie, nuklearmedizinischen Untersuchungen, Magnetresonanztomographie, Koronarangiographie, endomyokardialer Biopsie und elektrophysiologischen Untersuchungen. Häufig sind die Befunde nicht zielführend, und erst das Ansprechen auf eine gezielte Therapie ist die einzige Bestätigung der Verdachtsdiagnose. Zu den therapeutischen Optionen zählen eine medikamentöse Behandlung mit Immunsuppressiva und eine spezifische kardiale Medikation sowie eine Schrittmacher- oder Defibrillatorimplantation. Bei Versagen dieser Therapieformen ist die Herztransplantation als Ultima Ratio eine Option.

Abstract

Sarcoidosis is a systemic granulomatous disease of unknown etiology that is histologically characterised by non-caseating granulomas. Hilar lymph nodes, lung, eyes and skin are the organs most commonly affected, although the heart, liver, spleen and bones may also be involved. Cardiac involvement is relatively rare and is associated with a poor prognosis. In addition to clinically manifest heart failure, the most common clinical manifestations are arrhythmias and sudden cardiac death. The absence of clinical symptoms does not rule out cardiac sarcoidosis, because most cases are detected as late as at necropsy. The diagnosis is based on a series of diagnostic approaches, such as electrocardiography, echocardiography, radionuclide imaging, magnetic resonance imaging, coronary angiography, endomyocardial biopsy and electrophysiological testing. However, the test results often do not allow the diagnosis to be made, and response to therapy is the only confirmation of the anticipated diagnosis. Various therapeutic modalities include treatment with immunosuppressive agents and specific cardiac medication, and implantation of a pacemaker or an implantable cardioverter defibrillator. If these methods fail to succeed, heart transplantation remains the therapy of choice.

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  1. Medizinische Klinik II, Rhein. Friedrich Wilhelms Universität Bonn, Universitätsklinikum Bonn, Sigmund-Freud-Straße 25, 53105, Bonn, Deutschland

    S. Pabst, I. Tuleta & C. Grohé

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Pabst, S., Tuleta, I. & Grohé, C. Kardiale Sarkoidose. Kardiologe 2, 299–311 (2008). https://doi.org/10.1007/s12181-008-0087-8

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