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Giant Cell Myocarditis is a rare but devastating disease
that usually 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.
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,
Cooper, et al, NEJM 1997). Post-transplantation survival is approximately 71% at
five years despite a 25% rate of giant cell infiltration in the donor heart. To
confirm these findings, a randomized trial of immunosuppression including
muromonab-CD3, cyclosporine, and steroid is underway. Participation in the
Multicenter Giant Cell Myocarditis Treatment Trial from medical centers with
experience in the diagnosis and treatment of Giant Cell Myocarditis is
encouraged. Futher information may be obtained from Dr. Leslie Cooper at the Mayo Clinic in Rochester Minnesota. |
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