Introduction: Cardiac Amyloidosis is a rapidly progressive infiltrative cardiomyopathy associated with significant mortality and morbidity. It is characterised by the deposition of amyloid fibrils, misfolded protein deposits, in the myocardium and there are two types described. One, light chain amyloidosis (AL), is due to myocardial deposition of monoclonal immunoglobulin light chain proteins produced by bone marrow plasma cells and is rare. The second, transthyretin amyloidosis (ATTR), is caused by a transport protein that is synthesized primarily by the liver. ATTR has two sub-types, hereditary or variant (vATTR) and wild-type (wtATTR), with wildtype being the significantly more prevalent of the two.
AL and ATTR have different clinical courses, prognosis, and potential treatments. Cardiac involvement is common in both, occurring in up to 75% of AL cases and it is the dominant clinical feature in ATTR patients. ATTR has a slower and more varied clinical course with a median survival of around 3-5 years if untreated, compared to the more rapid progression of AL, with an untreated median survival of 6 months.
