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Book

Transthyretin Amyloid Cardiomyopathy (ATTR-CM)

Anubhav Jain  1 , Farah Zahra  2
In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan.
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Book

Transthyretin Amyloid Cardiomyopathy (ATTR-CM)

Anubhav Jain et al.
Free Books & Documents

Excerpt

Transthyretin amyloid cardiomyopathy (ATTR-CM) is one of the types of systemic amyloidosis in which misfolded transthyretin (TTR) protein gets deposited in the myocardium. Another pertinent etiology of cardiac amyloidosis is due to the deposition of immunoglobulin light-chain (AL) aggregates. Several other amyloidogenic proteins may get deposited in various organs and tissues but rarely involve the myocardium.

Transthyretin amyloidosis (ATTR) is a systemic disease. Due to amyloid deposition in extracardiac tissues, patients often have associated extracardiac signs and symptoms. However, isolated cardiac involvement has been reported as well. Diagnosis of ATTR-CM was often missed or delayed due to previously lacking optimal diagnostic modalities. ATTR-CM often progresses to advanced stages with minimal clinical signs and symptoms initially and is therefore associated with poor prognosis.

With improving bone avid radiotracer scintigraphy technology and the advent of new therapeutic options, diagnosis and treatment of ATTR-CM have become possible. 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.

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Conflict of interest statement

Disclosure: Anubhav Jain declares no relevant financial relationships with ineligible companies.

Disclosure: Farah Zahra declares no relevant financial relationships with ineligible companies.

References

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