Cardiac amyloidosis overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aarti Narayan, M.B.B.S [2]; Raviteja Guddeti, M.B.B.S. [3]
Overview
Cardiac amyloidosis is a well-known progressive condition, which has been reported with varying incidence rates. It refers to extracellular deposition of light chains of some serum proteins, that assume a beta pleated structure.
Myocardial involvement results in congestive heart failure and fatal arrhythmias and is the leading cause of death in patients with amyloidosis. Systemic amyloidosis of AL and TTR type is often associated with amyloid deposition in heart valves, in addition to blood vessels and myocardium [1] [2] In this classical type of valvular amyloidosis are the deposits occur in previously unaltered valves.
There is also another type of cardiac amyloidosis restricted entirely to the heart valves (surgically removed for chronic valvular disease) or only the atria (also called isolated atrial amyloidosis). The deposits are small and restricted to the areas of scarring and calcification.[3] The amyloid protein has not been identified but it has been demonstrated not to be AL or AA [4] [5]
This condition is heavily underdiagnosed because the thickening of ventricles from extracellular deposition of amyloid material in the heart is mostly attributed to chronic hypertension, which is also a feature of cardiac amyloidosis.
References
- ↑ Pomerance, A. The pathology of senile cardiac amyloidosis. J. Pathol. Bacteriol. 91, 357. 1966
- ↑ Buja, L. M., Khoi, N. B., and Roberts W. C. Clinically significant cardiac amyloidosis. Clinicopathologic findings in 15 patients. Am. J. Cardiol., 26, 394. 1970
- ↑ Goffin, Y. A. Microscopic amyloid deposits in the heart valves: a common local complication of chronic damage and scarring. J. Clin. Pathol., 33, 262. 1980
- ↑ Goffin, Y. A., Murdoch, W., Cornwell, G. G. III, and Sorenson, G. D. Microdeposits of amyloid in sclerocalcific heart valves: a histochemical and immunoflourescence study. J. Clin. Pathol., 36, 1342. 1983
- ↑ Amyloid and amyloidosis. Gilles Grateau, Robert A. Kyle, Martha Skinner, 2005 ISBN 0-8493-3534-5