The most common infiltrative cardiomyopathy is amyloidosis,
which by electron microscopy consists of rigid, nonbranching
fibrils lying in between the myocardial muscle cells (see figures
74c,
74d,
77b).
There are two kinds of amyloidosis:
1) primary
in which there is no coexisting disease, except in some cases
of multiple myeloma (a disease of the plasma cell, which proliferates
out of control to crowd out the normal bone marrow cells resulting
in anemia and other abnormalities).
2)
secondary amyloidosis may be associated with chronic diseases
like tuberculosis, or rheumatoid arthritis. The heart becomes
stiff as a result of the infiltration of the amyloid into the
heart muscular tissue leading to restrictive heart mechanisms
of contraction.
The echocardiogram shows thickening of both the right and left
ventricular muscle (to see the entire photographs of figures
above
click on: 73a,
73b,
74a, 74b,
74c,
74d,
77a,
77b,
77c,
77d, 77e).
The prognosis is poor and most treatments are ineffective.
In addition to an EKG, chest x-ray, and Doppler
echocardiogram, cardiac catheterization is necessary to document
the diagnosis with special attention being focused on the venous
pressure, which is characteristically elevated considerably
above normal, with an initial dissent and then a plateau.
While the left systolic ventricular pressure
is normal,the left ventricular diastolic pressure shows the
same abnormalities as the right ventricular blood pressure (see
figures 74a,
77d,
77e).