Zusammenfassung
□ In der neuen Klassifikation der Kardiomyopathien der WHO/ISFC Task Force von 1995 findet sich erstmals der Begriff entzündliche Kardiomyopathie als Äquivalent einer (chronischen) Myokarditis mit Zeichen der kardialen Dysfunktion. Ebenso werden wie bei der akuten Myokarditis idiopathische, autoimmune und infektiöse Formen der entzündlichen Kardiomyopathie unterschieden.
□ Bei Untersuchung der Endomyokardbiopsien von Patienten mit Kardiomyopathien wurde ebenso wie bei Patienten mit Myokarditis der immunhistochemische Nachweis eines entzündlichen Infiltrates (>14 Lymphozyten/Makrophagen/mm3) gefordert, wobei hier die Kriterien der ISFC Task Force 1997 zugrunde gelegt wurden.
□ Zur weiteren Differenzierung der inflammatorischen Kardiomyopathie sind molekularbiologische Untersuchungen zum Nachweis von Virusgenom in der Endomyokardbiopsie unverzichtbar. Neben den Enterovirusinfektionen dürften Infektionen mit DNA-Viren, wie zum Beispiel Zytomegalie-, Adeno- und Epstein-Barr-Virus bzw. Borrelien eine wesentliche Rolle spielen. So konnten in der Vergangenheit gehäuft diese DNA-Viren in Endomyokardbiopsien von Patienten nachgewiesen werden.
□ In verschiedenen Studien wurde bei Patienten mit entzündlicher Kardiomyopathie eine Inzidenz für Zytomegalievirus-DNA von 10% und für Adenovirus-DNA in 17% der Fälle gezeigt. Der Nachweis von Borrelien gelang nur in wenigen Fällen (<1%). Bei Patienten mit dilatativer Kardiomyopathie ohne Entzündung lagen die entsprechenden Inzidenzen für Zytomegalievirus bei 12% und für Adenovirus bei 15%. Borrelien konnten hier nur in 0,5% der Fälle nachgewiesen werden.
□ Findet sich in der Endomyokardbiopsie eine entzündliche Kardiomyopathie ohne Viruspersistenz, kann eine immunsuppressive Therapie im Rahmen von Studien erfolgen. Bei Nachweis von Adeno- oder Zytomegalievirusgenom empfehlen wir die immunmodulatorische Behandlung mit Hyperimmunglobulinen bei Zytomegalievirus- bzw. Immunglobulinen bei Adenovirusmyokarditis im Rahmen einer doppelblind randomisierten Studie. Bei Borreliosenachweis in der Polymerasekettenreaktion oder bei klinischen und serologischen Hinweisen auf eine kardiale Borreliose (Lyme-Erkrankung) ist eine dreiwöchige Antibiotikatherapie mit einem Cephalosporin der dritten Generation indiziert.
Abstract
□ In the report of the 1995 WHO/ISFC task force on the definition and classification of cardiomyopathies a new entity within the dilated cardiomyopathies was introduced as “inflammatory cardiomyopathy”. It is defined as myocarditis associated with cardiac dysfunction. Idiopathic, autoimmune and infectious forms of inflammatory cardiomyopathy are now recognized through this definition. Dilated cardiomyopathy with inflammation (DCMi, chronic myocarditis) was also defined by a recent ISFC task force as >14 lymphocytes/macrophages/mm3. Enteroviruses, adenoviruses and cytomegaloviruses are considered as main etiopathogenetic factors in the pathogenesis of inflammatory heart disease and have been demonstrated as important trigger for inflammatory cardiac disease. They may also cause dilated cardiomyopathy by viral persistence or secondary immunopathogenesis due to antigenic or molecular mimicry. For the detection of viral persistance the investigation of endomyocardial biopsies in patients with cardiomyopathy by the use of polymerase chain reaction and southern blot analysis is an important step for the standardization of diagnostic criteria on virally induced inflammatory cardiomyopathy. Present studies indicate an incidence of cytomegalovirus-DNA in patients with inflammatory cardiomyopathy in 10%, adenoviral-DNA in 17% and borreliosis only in rare cases (<1%). In dilated cardiomyopathy without inflammation the respective incidences were for cytomegalovirus 12%, 15% for adenovirus and only 0.5% of cases for borreliosis.
□ In addition the results of immunohistochemical analysis and molecular biological investigations of endomyocardial biopsies may have implications for future therapeutic studies. Depending on the etiology of the disease, immunosuppression may have benefit for patients with virus-negative cardiomyopathy with inflammation in contrast to patients with cytomegalo-, adenovirus-DNA or enteroviral persistence, in whom immunomodulation with hyperimmunoglobulins or immunoglobulins may be a feasible therapeutic option. Patients with a positive PCR for Borrelia burgdorferi should be treated with 3rd generation cephalosporines and/or sublactam.
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Pankuweit, S., Hufnagel, G., Eckhardt, H. et al. Kardiotrope DNA-Viren und Bakterien in der Pathogenese der dilatativen Kardiomyopathie mit und ohne Entzündung. Med Klin 93, 223–228 (1998). https://doi.org/10.1007/BF03044797
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DOI: https://doi.org/10.1007/BF03044797
Schlüsselwörter
- Inflammatorische Kardiomyopathie
- Adenoviren
- Zytomegalieviren
- Borreliose
- Endomyokardbiopsie
- Polymerasekettenreaktion