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Updated: May 22, 2025
06:33
Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
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Cardiac Amyloidosis: How Its Epidemiology is Changing
Giorgia Panichella1, Alberto Aimo2, Giuseppe Vergaro2
1Department of Clinical and Experimental Medicine, University of Florence, Florence 50134, Italy.
Heart Failure Clinics
|March 13, 2025
Summary
Cardiac amyloidosis (CA) involves amyloid buildup in the heart. While immunoglobulin light chain CA is stable, wild-type transthyretin CA is increasing, impacting screening and management.
Area of Science:
- Cardiology
- Genetics
- Epidemiology
Background:
- Cardiac amyloidosis (CA) is a progressive condition caused by amyloid fibril accumulation in the heart.
- It leads to diastolic dysfunction, heart failure, and increased mortality.
- Two main types are immunoglobulin light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR).
Purpose of the Study:
- To review the current epidemiology of cardiac amyloidosis.
- To highlight the increasing incidence and prevalence of wild-type transthyretin CA.
- To discuss the implications for clinical screening and management strategies.
Main Methods:
- Literature review of epidemiological studies on cardiac amyloidosis.
- Analysis of incidence and prevalence data for different CA subtypes.
- Synthesis of findings to inform screening and management.
Main Results:
- Immunoglobulin light chain amyloidosis (AL) incidence is stable at 8-15.2 cases per million persons (PMP)/year.
- Wild-type transthyretin CA incidence is increasing (14-27 cases PMP/year).
- Wild-type transthyretin CA prevalence is estimated at 30-170 cases PMP.
Conclusions:
- The rising prevalence of wild-type transthyretin CA necessitates updated screening protocols.
- Early detection and management are crucial for improving patient outcomes.
- Further research into CA epidemiology is essential for effective public health strategies.
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