Amyloid diseases constitute a group of conditions of diverse causes characterized by the accumulation of ultrastructually fibrillar material in various tissues in quantities sufficient to compromise vital organ function.
The symptoms of the amyloidoses depend upon the amount and localization of the deposits.
Myocardial involvement with amyloid is a common cause of secondary restrictive cardiomyopathies caused by a variety of conditions (amyloid, sarcoid, Gaucher disease, Hurler disease). Of the three major functional categories of the
cardiomyopathies
(dilated, hypertropic and restrictive), the restrictive are the least common in Western countries. The hallmark of the restrictive cardiomyopathies is abnormal diastolic function with excessively rigid ventricular walls.
We had experienced one case of amyloidosis, especially involving heart who was admitted to Hangyang University Hospital due to dyspnea on exertion, abdominal distension and edema on both lower extremites.
Echocardiogram showed markedly thickened septum and ventricular wall, as wall, as well as mitral valve thickening, and left atrial enlargement. Doppler echocardiogram showed markedly increased E wave, and in pulmonary venous flow by
tranesophageal
echocardiogram. D wave was prominent. In tissue sections of endocardium and rectum, with conventional staining technique, amyloid appears homogenous and eosinophilic. Amyloid showed apple-green birefrigence with Congo-red stain and under
polarized
light. On electron microscopy of endocardial biopsy, typical rigid, nonbranching fibrils (amyloid fibrils) are arranged randomly and are noted in the interstitium and around blood vessels, surrounding cardiac myocytes and capillaries. Kappa light
chain
multiple myeloma was diagnosed by bone marrow aspiration, biopsy and immunoelectrophoresis of serum and urine. He was treated with mephalan, prednisolone and diuretics.
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