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Giant cell myocarditis

Diagnosis and treatment

Riesenzellmyokarditis

Diagnose und Therapie

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Abstract

Giant cell myocarditis (GCM) usually presents as acute dilated cardiomyopathy that does not improve with guideline-based treatments. Ventricular tachycardia and heart block occur in a substantial number of patients. Diagnosis by endomyocardial biopsy can allow for the addition of immunosuppressive therapy and timely use of mechanical circulatory support when indicated. Recent studies suggest that the ventricular arrhythmias in GCM may be mediated by a cytokine-induced change in desmosomal protein expression. Genomic and proteomic studies suggest that the regulation of inflammatory pathways differs in GCM from lymphocytic myocarditis. Transplantation remains an effective therapy despite a 20–25% risk of GCM recurrence in the allograft. Recurrence in the native heart occurs up to 8 years after initial diagnosis. The long-term management of GCM patients, who initially recover, is not known and highlights the need for continuing multicenter collaborative clinical investigations.

Zusammenfassung

Das klinische Bild der Riesenzellmyokarditis („giant cell myocarditis, GCM) entspricht einer dilatativen Kardiomyopathie, die gegenüber einer leitlinienbasierten Herzinsuffizienztherapie refraktär ist. Kammertachykardien und AV-Blockierungen finden sich in einem nicht zu unterschätzenden Teil der Patienten ebenfalls. Mittels Endomyokardbiopsie können die Diagnose gesichert und die Patienten einer immunsuppressiven Therapie und ggf. auch einer Therapie mit Assist-Devices und mechanischer Herzunterstützung zugeführt werden. Jüngste Untersuchungen zeigen, dass den Kammertachykardien eine zytokininduzierte Alteration der desmosomalen Proteinexpression zugrunde liegt und dass sich die Regulation der Inflammationskaskade von einer lymphozytären Myokarditis unterscheidet. Die Herztransplantation stellt eine effektive Behandlungsalternative dar, auch wenn sich in 20–25% der Fälle eine Riesenzellmyokarditis in den Transplantatherzen wiederfindet. Auch bei effektiv behandelten Patienten konnten bis zu 8 Jahre nach der Erstdiagnose Rezidive nachgewiesen werden. Zum Langzeitverlauf und zur Langzeitbehandlung der Riesenzellmyokarditis sind weitere Multizenterstudien unverzichtbar.

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On behalf of all authors, the corresponding author states that there are no conflicts of interest.

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Correspondence to L.T. Cooper Jr. MD.

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Cooper, L., ElAmm, C. Giant cell myocarditis. Herz 37, 632–636 (2012). https://doi.org/10.1007/s00059-012-3658-1

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  • DOI: https://doi.org/10.1007/s00059-012-3658-1

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