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==Pathophysiology==
==Pathophysiology==
Data from a Lewis Rat model and from observational human studies suggest that GCM is mediated by [[T lymphocytes]] and may respond to treatment aimed at attenuating T cell function.  
Data from a Lewis Rat model and from observational human studies suggest that giant cell myocarditis is mediated by [[T lymphocytes]].  The disease may therefore respond to treatment aimed at attenuating T cell function.  


Numerous autoimmune disorders have been associated with giant-cell myocarditis in case reports, but the true magnitude of the association and causality of any association (if any) is not clear.
Numerous autoimmune disorders have been associated with giant-cell myocarditis in case reports, but the true magnitude of the association and causality of any association (if any) is not clear.

Revision as of 14:05, 5 September 2011

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor: Cafer Zorkun, M.D., Ph.D. [2]

Synonyms and Keywords: GCM, Idiopathic Giant cell myocarditis, IGCM

Overview

Giant cell myocarditis is a rare but often fatal inflammatory process involving the myocardium. Other than cardiac transplantation, there is no known effective treatment for giant cell mycoarditis.

Pathophysiology

Data from a Lewis Rat model and from observational human studies suggest that giant cell myocarditis is mediated by T lymphocytes. The disease may therefore respond to treatment aimed at attenuating T cell function.

Numerous autoimmune disorders have been associated with giant-cell myocarditis in case reports, but the true magnitude of the association and causality of any association (if any) is not clear.

Gross Pathological Findings

Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology

Microscopic Pathological Findings

Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology

Natural History, Complications, and Prognosis

Post-transplantation survival is approximately 71% at five years despite a 25% rate of giant cell infiltration in the donor heart.

These findings should be confirmed with a randomized trial of immunosuppression including muromonab-CD3, cyclosporine, and steroids.

Giant cell myocarditis may recur after transplantation but may respond to augmented immunosuppression.

GCM is usually characterized by progressive congestive heart failure, and is frequently associated with refractory ventricular arrhythmias. The majority of patients die secondary to congestive heart failure, although some have survived for long periods, often after immunosuppressive treatment. [1] [2]

This disease often affects young otherwise healthy individuals. The rate of death or heart transplantation is approximately 70% at one year.

The Giant Cell Myocarditis Registry is a clinical and pathologic database from 63 cases of giant cell myocarditis gathered from 36 medical centers. Findings from the registry include the following: the sensitivity of endomyocardial biopsy for GCM for patients who undergo transplantation or autopsy is approximately 80%. Registry subjects who received cyclosporine and/or azathioprine, with steroid and sometimes muromonab-CD3 had prolonged transplant-free survival (12.6 months vs 3.0 months for no immunosuppression.[3].

References

  1. Desjardins V, Pelletier G, Leung TK, Waters D. Successful treatment of severe heart failure caused by idiopathic giant cell myocarditis. Can J Cardiol 1992;8:788-92
  2. Ren H, Poston RS Jr, Hruban RH, Baumgartner WA, Baughman KL, Hutchins GM. Long survival with giant cell myocarditis. Mod Pathol 1993;6:402-7.
  3. Cooper LT, Berry GJ, Shabetai R.Idiopathic Giant-Cell Myocarditis — Natural History and Treatment. N Engl J Med. 1997 Jun 26;336(26):1860-6


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