Transthyretin amyloid cardiomyopathy (ATTR-CM) is a severe and often underestimated disease that impacts the heart muscle, potentially leading to fatal outcomes. In ATTR-CM, a protein called transthyretin, which normally circulates in the bloodstream, undergoes a structural transformation and accumulates in the heart, nerves, and other organs. According to the American Heart Association (AHA), the buildup of amyloid deposits in the heart can cause the walls to stiffen, resulting in impaired relaxation and filling of the left ventricle, a condition known as cardiomyopathy. As the disease advances, the heart may struggle to effectively pump blood out, ultimately resulting in heart failure. There are two main types of ATTR-CM: hereditary ATTR-CM (hATTR-CM) and wild-type ATTR-CM (wATTR-CM).
This article aims to provide insights into the most recent guidelines regarding ATTR-CM. While specific guidelines for ATTR-CM are currently unavailable, there are established guidelines for various forms of cardiac amyloidoses. Additionally, we will discuss the three most recent medications that have been approved for the treatment of ATTR-CM. The focus of this article will be on the guidelines and recent updates from the FDA concerning this condition.
Part 1 - Current Guidelines
To start, here is a listing of the cardiac amyloidosis guidelines published in the last five years:
- Comprehensive Multidisciplinary Care for the Patient With Cardiac Amyloidosis
- American College of Cardiology
- Publication: January 23, 2023
- Management of Heart Failure
- American College of Cardiology, American Heart Association, and Heart Failure Society of America
- Publication: March 31, 2022
- Multimodality Imaging in Cardiac Amyloidosis: Evidence Base and Standardized Methods of Imaging
- American Heart Association, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, and Society of Nuclear Medicine and Molecular Imaging
- Publication: June 30, 2021
- Multimodality Imaging in Cardiac Amyloidosis: Diagnostic Criteria and Appropriate Utilization
- American Heart Association, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, and Society of Nuclear Medicine and Molecular Imaging
- Publication: June 30, 2021
- Multimodality Imaging in Cardiac Amyloidosis: Part 1 of 2—Evidence Base and Standardized Methods of Imaging
- Society of Nuclear Medicine and Molecular Imaging
- Publication: August 29, 2019
- Multimodality Imaging in Cardiac Amyloidosis: Part 2 of 2—Diagnostic Criteria and Appropriate Utilization
- Society of Nuclear Medicine and Molecular Imaging
- Publication: August 29, 2019
Part 2 - Recent FDA Approvals
Below, is the medication recently approved by the FDA that we anticipate being incorporated into future US guidelines:
- ATTRUBY™ (acoramidis)
- BridgeBio, Inc.
- FDA approved for ATTR-CM on November 22, 2024
- ATTRUBY is a transthyretin stabilizer indicated for the treatment of the cardiomyopathy of wild-type or variant transthyretin-mediated amyloidosis (ATTR-CM) in adults to reduce cardiovascular death and cardiovascular-related hospitalization.
- VYNDAQEL® (tafamidis meglumine) and VYNDAMAX™ (tafamidis)
- Pfizer Inc.
- FDA approved for ATTR-CM on May 3, 2019
- VYNDAQEL and VYNDAMAX are indicated for the treatment of the cardiomyopathy of wild-type or hereditary transthyretin-mediated amyloidosis in adults to reduce cardiovascular mortality and cardiovascular-related hospitalization.
Thank you for taking the time to review the current ATTR-CM guidelines, as well as the recent major changes and FDA approvals that have recently occurred. We value your feedback and would like to hear your suggestions for future topics to be covered in our guideline series. Please feel free to contact us with any ideas or questions you may have.
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