Cardiac Amyloidosis

F1.largeCardiac amyloidosis is a manifestation of one of several systemic diseases known as the amyloidoses. This uncommon disease is often under diagnosed, as there are several types of amyloids that medical research has been able to identify and each has their own unique features and methods of treatment. Therefore, it is often unrecognized which can lead to errors in management of patient care. However, there is one identifying feature and that is the extracellular deposition of a proteinaceous material. When the deposit is stained with Congo red, it demonstrates an apple-green birefringence under polarized light and has a distinct color when stained with sulfated Alcian blue.

In addition these amyloid deposits, when viewed with electron microscopy, are seen to be composed of a β-sheet fibrillar material. These nonbranching fibrils have a diameter of 7.5 to 10 nm and are the result of protein misfolding. Cardiac involvement in amyloidosis (which can range from no signs to severe signs) is derived from a light-chain precursor (AL amyloidosis). Secondary amyloidosis almost never affects the heart in any clinically significant manner. The specific composition of the fibrils differs in the different types of amyloid. Both on the basis of common usage and for the sake of simplicity, “cardiac amyloidosis” is used here to describe involvement of the heart by amyloid deposition, whether as part of systemic amyloidosis (as is most commonly the case) or as a localized phenomenon. The complete results gathered from medical researches can be found from our source below.

Source

http://circ.ahajournals.org/content/112/13/2047.full