Suspected Cardiac Amyloidosis in a 67 year Old, Caucasian Female
Journal: International Journal of Pharmaceutical Sciences and Medicine (IJPSM) (Vol.5, No. 3)Publication Date: 2020-03-30
Authors : Georgiana Farrugia Bonnici; Analise Zarb; Mary Louise Camilleri; Paul John Cassar;
Page : 1-11
Keywords : amyloidosis; cardiac; amyloid; cardiac imaging; heart;
Abstract
Amyloidosis is a rare set of diseases that is characterised by the deposition of pathological amyloid in tissues and/or organs. Amyloid is a proteinaceous substance, 95% of which consists of aggregates of misfolded, insoluble proteins; that make up fibrillar material, and the remaining 5% consists of the P component, (a pentameric protein) and other glycoproteins. The heart is commonly involved in amyloidosis and can occur as part of systemic amyloidosis or as localised disease (Firkle, et al., 2013). In cardiac amyloidosis, amyloid is deposited in the extracellular space of the heart, resulting in stiffening of the heart, with involvement of both ventricles and atria; as well as the valves, leading to the development of a restrictive cardiomyopathy. Cardiac involvement is associated with a poor prognosis. In the past, the diagnosis of cardiac amyloid was often made during autopsy, (Banypersad, et al., 2012; Falk, et al., 2016); however, advances in cardiac imaging have recently led to increased detection rates (Dungu, 2015). In this case report, we will describe the presentation, investigation and management of a 67 year old Caucasian female, with suspected cardiac amyloidosis.
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Last modified: 2020-03-23 16:05:52