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Cardiac amyloidosis
Background
Overview
Definition
Cardiac amyloidosis refers to infiltration of the heart muscle by amyloid proteins, resulting in cardiomyopathy.
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Pathophysiology
Cardiac amyloidosis is caused by extracellular accumulation of misfolded protein fragments (immunoglobulin light-chain and transthyretin protein) in the heart muscle.
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Disease course
Clinical manifestations are related to the development of restrictive cardiomyopathy and include angina, myocardial infarction, and HF. An increased risk of thromboembolic complications is also present.
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Prognosis and risk of recurrence
Unadjusted 5-year mortality rates are estimated at 65% in patients with AL cardiac amyloidosis and 44% in patients with ATTR cardiac amyloidosis.
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Guidelines
Key sources
The following summarized guidelines for the evaluation and management of cardiac amyloidosis are prepared by our editorial team based on guidelines from the European Society of Cardiology (ESC 2023,2021), the Heart Failure Society of America (HFSA/AHA/ACC 2022), the Canadian Heart Failure Society (CHFS/CCS 2020), the American Heart Association (AHA/HRS/ACC 2019), the Amyloidosis Research Consortium (ARC 2019), and the Heart Rhythm ...
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