Cardiac amyloidosis is a disorder caused by amyloid fibril deposition in the extracellular space of the heart. It can present with cardiac signs or symptoms or may be diagnosed as the result of screening in patients who manifest extracardiac signs of amyloidosis. The approach to diagnosis depends upon the clinical presentation and the results of initial testing.
Amyloidosis refers to the extracellular deposition of fibrils arising from a variety of serum proteins. These fibrils adopt a beta-pleated sheet configuration that leads to characteristic histologic changes. Amyloid deposits can occur in a variety of organs, but morbidity and mortality from amyloid deposition most commonly occur when the heart, kidney, liver, or autonomic nervous system are affected.