Giant cell myocarditis
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Definition
Giant cell myocarditis (GCM) or Idiopathic Giant cell myocarditis (IGCM) was first described in 1905. It is fairly rare; however, it is often fatal and there is no proven cure because of the unknown nature of the disorder. 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] [1]
This disease often affects young otherwise healthy individuals. The rate of death or heart transplantation is approximately 70% at one year. 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.
Numerous autoimmune disorders have been associated with giant-cell myocarditis in case reports, but no data have been available on the incidence of these disorders in a study population with giant-cell myocarditis.
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.[1].
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.
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
 Giant Cell Myocarditis: Gross dilated LV with focal fibrosis |
 Giant Cell Myocarditis: Gross dilated LV with focal fibrosis |
 Giant Cell Myocarditis: Gross dilated LV with focal fibrosis |
Giant cell myocarditis may recur after transplantation but may respond to augmented immunosuppression.
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
 Boecks Sarcoid Giant Cell Myocarditis |
 Boecks Sarcoid Giant Cell Myocarditis |
 22-year-old man was seropositive for HIV-1 and died suddenly. There was widespread inflammation of the myocardium with giant cells, resembling giant cell myocarditis, that may have represented a precursor to lymphoma. |
References
Additional Reading
- Moss and Adams' Heart Disease in Infants, Children, and Adolescents Hugh D. Allen, Arthur J. Moss, David J. Driscoll, Forrest H. Adams, Timothy F. Feltes, Robert E. Shaddy, 2007 ISBN 0781786843
- Hurst's the Heart, Fuster V, 12th ed. 2008, ISBN 978-0-07-149928-6
- Willerson JT, Cardiovascular Medicine, 3rd ed., 2007, ISBN 978-1-84628-188-4
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
