Update on the diagnostic and therapeutic approach to transthyretin cardiac amyloidosis: an underestimated entity
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Lopez Barrios VM, Chacón Quirós MM, Apuy Rodríguez F, Sáenz Madrigal ME. Update on the diagnostic and therapeutic approach to transthyretin cardiac amyloidosis: an underestimated entity. Rev.méd.sinerg. [Internet]. 2024Aug.1 [cited 2024Sep.26];9(8):e1150. Available from: https://revistamedicasinergia.com/index.php/rms/article/view/1150

Abstract

Cardiac amyloidosis (CA) is a serious condition that affects heart function due to the abnormal deposition of amyloid. It can be caused by different types of proteins, with the most common being light chains (AL) and transthyretin (ATTR) in its hereditary (ATTRv) and non-hereditary (ATTRwt) forms. The ATTRwt variant is the most prevalent, and its early diagnosis poses a challenge due to the variability of clinical manifestations and the lack of specific diagnostic tests. Thus, the classic phenotype of CA is heart failure with preserved ejection fraction (HFpEF). Diagnostic suspicion is based on clinical, electrocardiographic, and echocardiographic findings; with magnetic resonance imaging, bone scintigraphy with bisphosphonates, and determination of monoclonal components in blood and/or urine reserved for confirmation. Additionally, cardiac rhythm disorders, syncope, and aortic valve disease are also frequent manifestations of CA. Treatment involves managing cardiac complications (supportive care) and the amyloidogenic process (specifically). It is worth noting that recent advances in diagnostic processes and new therapies available translate into improvements in patient prognosis. This review aims to synthesize the diagnostic and therapeutic approach to ATTR-CA.

https://doi.org/10.31434/rms.v9i8.1150

Keywords

cardiac amyloidosis. transthyretin. heart failure. diagnosis. treatment.
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