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February 17, 2O23

ACC releases 2023 Expert Consensus on Cardiac Amyloidosis

 Cardiac amyloidosis biopsy findings (K.et al., 2013)

Cardiac amyloidosis biopsy findings (K.et al., 2013)

The American College of Cardiology (ACC) published online the 2023 ACC Expert Consensus Decision Pathway on Comprehensive Multidisciplinary Care for the Patient with Cardiac Amyloidosis.  

Cardiac amyloidosis is a type of restrictive cardiomyopathy (CM) that occurs when amyloid, an abnormal protein, builds up in the heart. Amyloid replaces normal cardiac tissue, interfering with the conduction of electrical signals inside the heart, leading to abnormal heartbeats (arrhythmia). As more amyloid accumulates, the heart struggles to pump efficiently, and if left untreated, it can lead to heart failure.

Cardiac amyloidosis can be hereditary, can develop on its own (wild type), or progress from other diseases (cancer) or treatment (dialysis).  

While amyloidosis is not curable, it is treatable. For treatment to be effective in preventing loss of organ function, an early diagnosis is crucial. The 51-page consensus on cardiac amyloidosis highlights the role of an early diagnosis and provides a diagnostic algorithm for physicians to follow.  The diagnostic algorithm includes the use of monoclonal protein screen, scintigraphy, biopsy*, as well as pitfalls to avoid when deciding on which diagnostic tool to use.

The writing committee emphasizes the need for a multidisciplinary collaboration since amyloid fibrils can deposit in multiple organs – including the brain, intestines and kidneys. In addition, the document also includes implementation steps for amyloidosis treatment and the role of traditional arrhythmia and heart failure management.  

The published consensus document reflects current advances in diagnostic methods and effective therapies, but the writing group anticipates that the algorithms presented will continue to evolve with new evidence.  

Cardiac amyloidosis is often underdiagnosed and underrecognized so this comprehensive document aims to address the needs and improve the clinical outcomes for patients with cardiac amyloidosis.

References:

1. Kittleson, M. M., Ruberg, F. L., Ambardekar, A. V., Brannagan, T. H., Cheng, R. K., Clarke, J. O., Dember, L. M., Frantz, J. G., Hershberger, R. E., Maurer, M. S., Nativi-Nicolau, J., Sanchorawala, V., & Sheikh, F. H. (2023). 2023 ACC Expert Consensus Decision Pathway on Comprehensive Multidisciplinary Care for the Patient With Cardiac Amyloidosis. Journal of the American College of Cardiology. https://doi.org/10.1016/j.jacc.2022.11.022

2. K., G., Feng, D., Grogan, M., Taub, C., Dispenzieri, A., & W., K. (2013). Cardiac Amyloidosis: Typing, Diagnosis, Prognosis and Management. Amyloidosis. doi: 10.5772/53763

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