What causes cardiac amyloidosis?

Updated: Mar 25, 2020
  • Author: Gyanendra K Sharma, MD, FACC, FASE; Chief Editor: Terrence X O'Brien, MD, MS, FACC  more...
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Answer

Primary amyloidosis (AL) is a type of plasma cell dyscrasia and is the most common type involving the heart.

Secondary systemic amyloidosis seen in chronic inflammatory conditions rarely involves the heart. Organ dysfunction is usually reversible with resolution of the underlying inflammatory disorder.

Familial or hereditary amyloidosis occurs because of a mutation in the transthyretin (TTR) gene located on the chromosome 18. Most patients with ATTR have heterozygous mutation and autosomal dominant inheritance.

Senile systemic amyloidosis, also known as wild-type transthyretin (wt-TTR), is common in people over 70 years of age and has a better prognosis.


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