Cardiac amyloidosis. Therapeutic and diagnostic difficulties with reference to two different forms of the disease.

Abstract

Two male patients with primary cardiac amyloidosis are described. Patient 1 presented with typical effort angina pectoris with no ischemic electrocardiographic changes and a normal coronary angiogram. At necropsy, a severe diffuse, intravascular amyloid deposition was observed in the intramural coronary arteries. In patient 2 the presenting symptom was congestive heart failure with echocardiographic evidence of asymmetric septal hypertrophy and pericardial effusion. Technetium-99m pyrophosphate scintigraphy showed diffuse myocardial uptake, and the diagnosis of cardiac amyloidosis was confirmed in the postmortem examination. The diagnostic and therapeutic problems associated with cardiac amyloidosis are discussed in the light of these case reports.