It is primarily synthesized by the liver (>95%). Transthyretin (previously known as prealbumin) is a normal circulating protein that functions as a carrier protein for retinol (vitamin A) and thyroxine. As diagnostic capabilities are getting better, studies have shown a higher prevalence of ATTR-CM in patients with heart failure with preserved ejection fraction than previously perceived. With improving bone avid radiotracer scintigraphy technology and the advent of new therapeutic options, diagnosis and treatment of ATTR-CM have become possible. ATTR-CM often progresses to advanced stages with minimal clinical signs and symptoms initially and is therefore associated with poor prognosis. Diagnosis of ATTR-CM was often missed or delayed due to previously lacking optimal diagnostic modalities. However, isolated cardiac involvement has been reported as well. Due to amyloid deposition in extracardiac tissues, patients often have associated extracardiac signs and symptoms. Transthyretin amyloidosis (ATTR) is a systemic disease. Several other amyloidogenic proteins may get deposited in various organs and tissues but rarely involve the myocardium. Another pertinent etiology of cardiac amyloidosis is due to the deposition of immunoglobulin light-chain (AL) aggregates. Transthyretin amyloid cardiomyopathy (ATTR-CM) is one of the types of systemic amyloidosis in which misfolded transthyretin (TTR) protein gets deposited in the myocardium.

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