ABSTRACT
Amyloidosis results from the extracellular deposition of amyloid fibrils, insoluble structures comprised of misfolded proteins, in the heart, kidneys, liver, gastrointestinal tract, peripheral nerves, and other soft tissues. The two proteins that most frequently infiltrate the heart are the immunoglobin light chains (AL amyloidosis) and transthyretin (ATTR amyloidosis). ATTR amyloid cardiomyopathy is increasingly recognized as a cause of heart failure, particularly heart failure with preserved ejection fraction. There have been many advancements in chemotherapy for the treatment of AL amyloidosis and new developments regarding transthyretin-targeted therapies for ATTR amyloidosis; however, advanced heart failure therapies may still need to be considered in patients with end-stage heart failure secondary to amyloid cardiomyopathy.
