Abstract
Background: Giant cell myocarditis causes essentially irreversible fulminant left ventricular
dysfunction with associated conduction abnormalities and congestive failure. Response
to immunosuppressive therapy is poor and cardiac transplantation is the only viable
treatment option. The histologic hallmarks of giant cell myocarditis include a polymorphous
inflammatory response with numerous multinucleated giant cells and extensive myocyte
necrosis in a geographic pattern. There were 38 patients who received a cardiac transplant
for giant cell myocarditis in the Giant Cell Myocarditis Registry. Among these patients,
there were 9 recurrences of disease in the allograft. Concern has been expressed that
recurrence of giant cell myocarditis in the allograft might be a contraindication
for cardiac transplantation in the future.
Methods
In our single-center analysis we describe the clinical and histologic findings of
5 patients transplanted for giant cell myocarditis at the Cleveland Clinic.
Results
All but 1 of the patients were New York Heart Association (NYHA) class 4 with an average
cardiac index (CI) of 1.52 liters/min · m2. Of the 5 patients transplanted, 1 developed recurrent giant cell myocarditis. Routine
right ventricular endomyocardial biopsy at 1 week exhibited severe multifocal myocardial
fibrosis in addition to mild acute vascular rejection and mild grade 1A cellular rejection.
Follow-up biopsy in this patient indicated grade IIIA moderate acute rejection in
addition to multinucleated giant cells. Two distinct inflammatory processes were noted
consisting of foci of T-cell inflammation identified by immunohistochemistry to be
consistent with rejection, and a second inflammatory process with few mononuclear
cells staining for macrophage or T-cell markers with eosinophils and myocyte necrosis
consistent with giant cell myocarditis. Follow-up right ventricular endomyocardial
biopsies (RVBXs) in this patient have subsequently demonstrated improvement in the
degree of inflammatory infiltrate without vascular or significant cellular rejection.
Vascular rejection was noted in 1 of the remaining 4 patients and was treated successfully
with muramab-CD3 and plasmapheresis.
Conclusions
Giant cell myocarditis should be expected to recur in the allograft and often does
so concurrently with rejection. However, the disease in the allograft responds to
therapy in a favorable manner, which differs dramatically from that in the native
heart. This might be the result of detection of the disease at an earlier stage than
in the native heart, or the immunosuppression milieu in the allograft. The favorable
response to therapy suggests that the likelihood of recurrence of giant cell myocarditis
should not be considered a barrier to transplantation.
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References
- Giant cell myocarditis.J Heart Lung Transplant. 1995; 14: 394-401
Cooper LT, Berry GJ, Shabetai R, for the Multicenter Giant Cell Myocarditis Study group investigators. N Engl J Med 1997;336:1860–6.
- Giant cell myocarditis.J Heart Lung Transplant. 1992; 11: 370-374
- Eosinophilic explant carditis with eosinophilia.J Heart Lung Transplant. 1995; 14: 755-760
- Hypersensitivity myocarditis in transplant candidates.J Heart Lung Transplant. 1991; 10: 688-697
Cooper LT, Olson LJ, Berry GJ, Tazelaar H. Post-transplantation survival of patients with idiopathic giant cell myocarditis (GCM) versus cardiomyopathy. J Am Coll Cardiol 1998;31:251A, Abstract #1097-55.
Article info
Publication history
Accepted:
March 9,
1999
Received:
April 14,
1998
Identification
Copyright
© 2001 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.
