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Amyloid and the Heart

  • Heart Failure (HJ Eisen, Section Editor)
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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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Funding

Jignesh Patel reports grants from the National Institutes of Health (Grant number: 1UO1AI136816-01).

Author information

Authors and Affiliations

  1. Sarver Heart Center, University of Arizona, 1501 North Campbell Avenue, Tucson, AZ, 85724, USA

    Aaron M. Wolfson

  2. University of Utah Health Sciences Center, 30 North 1900 East, Rm 4A!00, Salt Lake City, UT, 84132, USA

    Kevin S. Shah

  3. Heart Transplant Program, Cardiac Amyloidosis Program, 8536 Wilshire Blvd, 3rd floor, Beverly Hills, CA, 90211, USA

    Jignesh K. Patel

  4. Smidt Cedars-Sinai Heart Institute, 127 S San Vicente Blvd, Los Angeles, CA, 90048, USA

    Jignesh K. Patel

Authors
  1. Aaron M. Wolfson
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  2. Kevin S. Shah
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  3. Jignesh K. Patel
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Correspondence to Jignesh K. Patel.

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Aaron M. Wolfson and Kevin S. Shah declare that they have no conflict of interest.

Jignesh Patel reports grants, personal fees, non-financial support and other from Alnylam Pharmaceuticals; personal fees, non-financial support and other from Akcea Pharmaceuticals; grants, personal fees, non-financial support and other from Pfizer Inc.; grants from Ionis Pharmaceuticals; grants from Alexion Pharmaceuticals; grants from CareDx Inc., and grants from Genzyme Inc.

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Wolfson, A.M., Shah, K.S. & Patel, J.K. Amyloid and the Heart. Curr Cardiol Rep 21, 164 (2019). https://doi.org/10.1007/s11886-019-1230-9

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  • DOI: https://doi.org/10.1007/s11886-019-1230-9

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