preview

Amyloidosis Research Paper

Decent Essays
Open Document

Amyloidosis is a disease characterized by the extracellular deposition of insoluble fibrillar proteins in the tissues and organs including the heart [1,2]. There are 3 major subtypes of cardiac amyloid: light chain amyloidosis, hereditary transthyretin amyloidosis and wild-type transthyretin amyloidosis, also called senile systemic amyloidosis (SSA). SSA is almost exclusively a disease of the elderly caused by the deposition of wild-type transthyretin fibrils predominantly in the myocardium leading to progressive heart failure, arrhythmia and conduction blocks [3]. Post mortem studies have shown that the finding of amyloid depositis (wild-type transthyretin) is not uncommon [4], however the actual prevalence is not known.

The natural history of SSA is not well documented, most likely due to the fact that it’s rarely diagnosed before death [5]. Typically it presents with symptoms and signs of biventricular cardiac failure …show more content…

This, however, is difficult in the patient population affected, as an endomyocardial biopsy is an invasive procedure that patients are not keen to undergo. Cardiac MRI is a useful investigation when cardiac amyloid is suspected but it is unable to differentiate between different amyloid types [13]. Tc-DPD scintigraphy is a recent addition to the workup of the condition and has been shown to be a specific imaging tool for transthyretin cardiac amyloid [12].

The prognosis of patients with SSA is much better than other subtypes of cardiac amyloid [5,9]; a previous study found that for patients with SSA and heart failure the median survival was 5 years [9] compared with 6 months after the onset of heart failure for AL amyloidosis [14]. Currently no specific disease modifying therapies are available, although promising new agents are being investigated in a number of clinical

Get Access