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Published in: Current Cardiology Reports 12/2019

01-12-2019 | Cardiomyopathy | Heart Failure (HJ Eisen, Section Editor)

Amyloid and the Heart

Authors: Aaron M. Wolfson, Kevin S. Shah, Jignesh K. Patel

Published in: Current Cardiology Reports | Issue 12/2019

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Abstract

Purpose of Review

While morbidity and mortality remain high for amyloid cardiomyopathy (AC), increased awareness, earlier diagnosis, and advances in treatment have improved patient outcomes. This review will discuss the pathophysiology, contemporary diagnostic strategies, and novel and investigational therapeutic strategies for light-chain (AL) and transthyretin (ATTR) AC.

Recent Findings

Diagnostic strategies for AC now include cardiac magnetic resonance imaging and bone scintigraphy. Proteosome inhibitor therapy is now front-line therapy for AL AC followed by autologous stem cell transplantation. Emerging disease-modifying strategies for ATTR AC include the recently FDA-approved TTR-stabilizer, tafamadis. ATTR gene-silencing therapy and amyloid fibril degradation therapy are two other strategies under investigation. Heart transplantation and durable mechanical circulatory support remain a final potential option; however, contemporary outcomes are improving with better patient selection.

Summary

Patient outcomes for AC are expected to improve as increased awareness leads to earlier diagnosis and prompt treatment with emerging pharmacotherapy or advanced heart therapies.
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Metadata
Title
Amyloid and the Heart
Authors
Aaron M. Wolfson
Kevin S. Shah
Jignesh K. Patel
Publication date
01-12-2019

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