Although the heart may be involved in all forms of amyloidosis, cardiac amylodosis is most commonly a result of immunocyte disease (and resultant AL fibril deposition). Clinical cardiac abnormality is also associated with deposition of AA fibrils in familial Mediterrranean fever.
It is often an incidental post-mortem finding in an elderly patient (>50% of those over 70 yrs have cardiac amyloid). Deposits are usually widely spaced but when they occur subendocardially there is a risk of conducting tissue damage and arrhythmias.
Cardiac amyloidosis is a cause of restrictive cardiomyopathy.
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Cardiovascular involvement of amyloidosis is present in 90% of cases, which is frequently associated with the primary form of the disease (AL amyloidosis).
The prognosis of patients with systemic amyloidosis (AL amyloidosis) is directly associated with the presence of cardiac involvement, such that survival does not usually exceed 4 months after the onset of heart failure signs and symptoms (1)
Transthyretin amyloid (aTTR) cardiomyopathy
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