Heart involvement by
amyloid deposition remains the most challenging of all organ sytems that may become involved, in what is generally a systemic disease. The correct diagnosis of
amyloid type is critical to selection of the appropriate and wide range of
therapies. The treatment of
amyloid heart disease comprises two strategies: conventional management of a
restrictive cardiomyopathy, and varied
therapies aimed at the underlying amyloidogenic process. In light chain (
AL) amyloidosis, many of the most efficacious
therapies involve chemotherapeutic agents with their own inherent toxicities to the heart and bone marrow. In the case of the
hereditary amyloidosis, major surgery in the form of
liver transplantation is usually required. Moreover, consideration should be given to screening of family members for a potentially
hereditary disease. Several types of
amyloidosis may require one or more, of heart, liver and/or
kidney transplantation, sometimes in addition to high-dose
chemotherapy. The objective is to provide a schematic overview of available
therapies in the management of AL, hereditary, senile systemic, isolated atrial and secondary forms of
amyloidosis.