Associates' Presentations
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A case report of congestive heart failure from amyloidosis.
Harish Ponnuru, M.D., (Associate), Sinai Samaritan Medical Center
A case report of congestive heart failure from amyloidosis: a 76 year old female presents with dyspnea on exertion, shortness of breath, and a two week history of lower extremity edema. She underwent cardiac catheterization which revealed normal coronary arteries. A biopsy of the right ventricle was obtained at that time. Congo Red staining revealed apple-green birefringence when examined under polarized light microscopy, diagnostic for amyloidosis.
Discussion - Amyloid is a fibrillar protein, arranged in a betapleated sheet conformation. This structure confers resistance to phagocytosis and proteolysis and makes it relatively insoluble in vivo. The pathophysiology of the various forms of this disease relate directly to the particular tissue that accrues deposits of this protein. In primary amyloidosis, the AL (amyloid light chain) protein deposits systemically. A secondary form exists as well and is termed reactive systemic amyloid. This is due to the underlying presence of chronic disease or chronic hemodialysis. In this condition, AA (amyloid A) protein is deposited mostly in the liver, spleen, adrenals, kidney and intestinal tract.
Cardiac amyloidosis is associated with diffuse deposition in myocardial tissue, although it can be seen in pericardial tissue as well. As deposition increases throughout the ventricle, it become stiff and noncompliant. This leads to a picture of restrictive cardiomyopathy and symptoms of congestive heart failure. This is the most common clinical presentation of amyloidosis. If the coronary arteries themselves become involved, angina pectoris may develop. The ECG shows low voltage in half of patients. Deposition in conduction pathways leads to arrhythmia's with atrial fibrillation being most common. The echocardiogram demonstrates a sparkling granular texture. Biopsy of involved tissue is diagnostic. Treatment utilizing melphalan and prednisone does prolong survival, however, median survival with cardiac disease is only 5 months.
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