Entzündliche Herzmuskelerkrankung als Ursache einer Herzinsuffizienz

Abstract

Myocarditis is an inflammatory disease of the cardiac muscle caused by myocardial infiltration of immunocompetent cells following any kind of cardiac injury. Myocarditis is a challenging diagnosis due to the heterogeneity of clinical presentations. It can be caused by infections, drugs, toxic substances, and autoimmune diseases. Chronic inflammatory events may survive successful clearance of initial cardiotoxic agents, be triggered or amplified by autoimmunological processes, or develop in the context of systemic diseases. Progression of myocarditis to its sequela, dilated cardiomyopathy, has been documented in 25 % of cases and is pathogenically linked to chronic inflammation and viral persistence. Therapeutic decisions must be based on the results of endomyocardial biopsy studies while taking account of the individual patient's clinical course. Moreover, specific immunosuppressive and antiviral treatment can help only if a treatable cause is present (e. g., a viral infection, or inflammatory process), and only if the myocardium still has regenerative potential. Once irreversible myocardial injury has occurred then the development or progression of heart failure in the long term can no longer be prevented. The aims are to bridge the gap between clinical and tissue-based diagnosis, to improve management of aetiology-driven treatment in inflammatory heart muscle disease.

Kernaussagen

• Bei V. a. Myokarditis/inflammatorische Kardiomyopathie ist eine EMB klar indiziert.

• Eine exakte Analyse der EMB mittels Molekularbiologie, Histologie und Immunhistologie mit Charakterisierung und Quantifizierung von entzündlichen Infiltraten ist entscheidend.

• Eine spezifische Therapie stellt bei virusnegativen Patienten die Immunsuppression, bei Enterovirus-positiven Patienten die Interferon-b-Therapie dar.

• Bei fulminantem Verlauf mit V. a. Riesenzellmyokarditis ist die EMB eine Notfallindikation, eine Genexpressionanalyse bietet in diesem Fall ein neues diagnostisches Tool.

• Literatur

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