Amyloid disease of the heart

Cardiac amyloidosis ("stiff heart syndrome") occurs when amyloid deposits infiltrate the normal heart muscle, and is a cause of what is called a restrictive cardiomyopathy. However, a number of tests are not consistent with that diagnosis at this point (unless this is very early in the disease process). Severe left atrial enlargement can occur in association with hypertensionaortic stenosis, mitral incompetence, mitral stenosis and hypertrophic cardiomyopathy:

Cardiac ECHO of a patient with Amyloid disease, showing a severe restrictive cardiomyopathy. LA, RA normal sizel LV, RV walls severely thickned.

An Echocardiogram of severe left atrial enlargement and mitral stenosis:


The following tests may be performed to help diagnose cardiac amyloidosis:

Amyloidosis is often seen in people with multiple myeloma, a cancer of the plasma cells in the bone marrow. Senile amyloidosis is caused by overproduction of a different protein and occurs in older patients.