Int J Heart Fail.  2020 Oct;2(4):231-239. 10.36628/ijhf.2020.0016.

Untangling Amyloidosis: Recent Advances in Cardiac Amyloidosis

• Kim D
• Choi JO
• Kim K
• Kim SJ
• Jeon ES

• KMID: 2532601
• DOI: http://doi.org/10.36628/ijhf.2020.0016

Abstract

Cardiac amyloidosis (CA) is a highly underdiagnosed cause of heart failure. Amyloid lightchain (AL) and amyloid transthyretin (ATTR) cardiomyopathy are two major subtypes of cardiac amyloid. Amyloid fibril deposits cause cardiac dysfunction by mechanically infiltrating the myocardium or by direct cardiotoxicity. Achieving a timely diagnosis is important to initiate disease-modifying therapies and improve the survival of patients with CA. Therefore, physicians must be aware of “red flag symptoms” that increase suspicions for CA when assessing heart failure patients. Although endomyocardial biopsy is a definitive diagnostic tool, with recent advances in non-invasive imaging, non-biopsy diagnosis is feasible in ATTR CA. There have been major advances in treatments for both AL and ATTR CA, and survival of CA has improved. In addition to general management of heart failure, numerous treatment options are increasing for both AL and ATTR CA. Given the systemic nature of amyloids, multi-disciplined team approaches are crucial to management of CA. With recent development of diagnosis and treatment options for both AL and ATTR amyloidosis, it is no longer considered a non-treatable disease.

Keyword

Heart failure; Amyloidosis; Amyloid