Therapy of RCM in Specific Cases:
> Amyloidosis: has a poor prognosis, particularly in the presence of a monoclonal light chain in serum or urine, multiple myeloma, and hepatic involvement.
Senile systemic amyloidosis has a better prognosis. Untreated patients have a median survival of less than 6 months after the onset of heart failure. Median survival is less than 50% in 2 years despite therapy with melphalan, steroids, immunomodulating agents (lenalidomide and/or bortezomib), and stem cell transplantation.
> Prognosis of idiopathic restrictive cardiomyopathy is better than in amyloidosis but relatively poor in children. Transplantation is the only therapeutic option in advanced cases.
> Cardiac sarcoidosis: Prednisone (30–80 md od) tapered over 6 months with or without additional immunosuppression with methotrexate, mycophenolate mofetil, ciclosporin or infliximab.
Half of the patients with cardiac sarcoidosis require an ICD and 25% of them need permanent pacing.
> Loffler’s endocarditis may respond to steroids at early stages. Surgical decortication may be required in advanced cases of endomyocardial fibrosis, but operative mortality is high (15–25%).
> Replacement of alpha-galactosidase reduces wall thickness in Fabry’s disease.