Medical Care: Symptomatic relief is achieved by routine cardiac therapy, including diuretics, digitalis, afterload reduction, and anticoagulation, as indicated by Weller et al and Parillo et al.
" Treatment with low-dose imatinib causing rapid regression of both eosinophilic proliferation and endomyocardiopathy is described by Cools et al, Vandenberghe et al, and Rotoli et al.
" Early phases of the disease have been treated with immune suppressant and cytotoxic medications with varying degrees of success.
" Corticosteroids appear to be beneficial in acute myocarditis, as reported by Uetsuka et al, among others. Together with cytotoxic drugs, including hydroxyurea, corticosteroids may prolong survival substantially, as reported by Weller et al, Parillo et al, and Arnold et al.
" Interferon therapy also has been reported by Butterfield et al as having some success.
Surgical Care: Once fibrosis ensues, surgical therapy may have a positive impact on palliation of symptoms.
" Dubost et al performed the first endocardiectomy in endomyocardial fibrosis, which consists of decorticating the fibrosed endocardium in a manner similar to resection of constricting densely fibrotic pericardium.
" In 150 published cases of surgical therapy of endomyocardial fibrosis and eosinophilic myocarditis, an operative mortality rate of 15-29% is reported, with AV block requiring a permanent pacemaker as a common complication.
" Endocardiectomy is directed toward the predominant location of the restrictive process.
" The mitral and tricuspid valves may be subject to replacement or repair, depending on the involvement of the subchordal apparatus.
" Early surgery is assisted by the fact that fibrous septa may not have extended into the adjacent myocardium.
" In advanced disease, surgical therapy is the only option.
" The extent to which fibrosis recurs postoperatively is not known.
DRUG TREATMENT :
Symptomatic relief is achieved by routine cardiac therapy including diuretics, digitalis, afterload reduction, and anticoagulation.
Early phases of the disease have been treated, with varying degrees of success, with immune suppressants, including steroids and interferon therapy, and cytotoxic medications, particularly hydroxyurea.
Corticosteroids appear to be beneficial in acute myocarditis, together with cytotoxic drugs, including hydroxyurea, and may prolong survival substantially. Interferon therapy has also been reported as having some success.
1. TYROSINE KINASE INHIBITORS : These agents inhibit tyrosine kinase, which, in turn, inhibit activation of intracellular pathways that can promote deregulated cell proliferation.
- IMATINIB
2. ANTIMETABOLITES, ANTINEOPLASTIC AGENTS :
- HYDROXYUREA
3. CORTICOSEROIDS :
- METHYLPREDNISOLONE
4. DIURETICS :
- BUMETANIDE
- FRUSEMIDE
5. CARDIAC GLYCOSIDES :
- DIGOXIN
6. ANGIOTENSIN CONVERTING ENZYME INHIBITORS :
- ENALAPRIL
7. INTERFERONS :
- INTERFERON ALFA 2A