ATTR-CM a systemic disease caused due to buildup of transthyretin fibrils in the myocardium. It is an abnormal deposits of misfolded transthyretin protein (amyloid) in the heart walls of left ventricle (heart’s main pumping chamber) causing abnormal heart beat. It may impulsive on the tongue and cause tongue enlargement or it gathers in the muscles and narrow down the muscles. Deposit on myocardial wall may thicken more than 14 mm. As per recent studies, approximately 20% of it occur with heart failure. It can affect any person, but studies have shown elderly males are more prone to it as compared to youngsters.
There are 2 types of ATTr-cM:
Hereditary: A variant, caused by mutation in the transthyretin gene. It may occur at age of 50-60. Structural changes in the transthyretin protein can misfold and start depositing in various tissues, especially on myocardium and peripheral nerves. Habitually this type of ATTRCM develops to advanced stages with very less symptoms.
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