The term ‘amyloidosis ’ describes a group of disorders characterized by abnormal protein folding, leading to the aggregation and accumulation of these misfolded proteins, or ‘amyloid fibrils’ in the tissues of affected organs. These amyloid fibrils display the pathognomonic property of apple-green birefringence when viewed under cross polarized light after staining with Congo red. 1 The deposit of these insoluble proteins into tissues leads to marked interstitial expansion, direct cell toxicity and cell death.
In case of heart involvement, amyloidosis is associated with a particularly high morbidity and mortality. 2.3 Although there are more than 30 known amyloidogenic proteins, only 9 accumulate in the myocardium, and the two most frequent etiologies of cardiac amyloidosis (CA) are misfolding of either the transthyretin tetramer (ATTR) or immunoglobulin light-chains secreted by clonal plasma cells (AL), which combined account for 95% of cases of CA. 4
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