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Amyloidosis in Heart Failure

  • Comorbidities of Heart Failure (C Angermann, Section Editor)
  • Published:
Current Heart Failure Reports Aims and scope Submit manuscript

Abstract

Purpose

Amyloidosis represents an increasingly recognized but still frequently missed cause of heart failure. In the light of many effective therapies for light chain (AL) amyloidosis and promising new treatment options for transthyretin (ATTR) amyloidosis, awareness among caregivers needs to be raised to screen for amyloidosis as an important and potentially treatable differential diagnosis. This review outlines the diversity of cardiac amyloidosis, its relation to heart failure, the diagnostic algorithm, and therapeutic considerations that should be applied depending on the underlying type of amyloidosis.

Recent Findings

Non-biopsy diagnosis is feasible in ATTR amyloidosis in the absence of a monoclonal component resulting in higher detection rates of cardiac ATTR amyloidosis. Biomarker-guided staging systems have been updated to facilitate risk stratification according to currently available biomarkers independent of regional differences, but have not yet prospectively been tested. Novel therapies for hereditary and wild-type ATTR amyloidosis are increasingly available. The complex treatment options for AL amyloidosis are improving continuously, resulting in better survival and quality of life. Mortality in advanced cardiac amyloidosis remains high, underlining the importance of early diagnosis and treatment initiation.

Summary

Cardiac amyloidosis is characterized by etiologic and clinical heterogeneity resulting in a frequently delayed diagnosis and an inappropriately high mortality risk. New treatment options for this hitherto partially untreatable condition have become and will become available, but raise challenges regarding their implementation. Referral to specialized centers providing access to extensive and targeted diagnostic investigations and treatment initiation may help to face these challenges.

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Author notes

  1. Giovanni Palladini and Stefan Störk shared senior authorship

Authors and Affiliations

  1. Interdisciplinary Amyloidosis Center of Northern Bavaria, University Hospital Würzburg, Würzburg, Germany

    Sandra Ihne, Caroline Morbach & Stefan Störk

  2. Department of Internal Medicine II, Hematology, University Hospital Würzburg, Würzburg, Germany

    Sandra Ihne

  3. Comprehensive Heart Failure Center (CHFC), University and University Hospital Würzburg, Würzburg, Germany

    Sandra Ihne, Caroline Morbach & Stefan Störk

  4. Department of Internal Medicine I, Cardiology, University Hospital Würzburg, Würzburg, Germany

    Caroline Morbach & Stefan Störk

  5. Amyloidosis Research and Treatment Center, Fondazione IRCCS Policlinico San Matteo, V.le Golgi 19, 27100, Pavia, Italy

    Laura Obici & Giovanni Palladini

Authors
  1. Sandra Ihne
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  2. Caroline Morbach
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  3. Laura Obici
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  4. Giovanni Palladini
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  5. Stefan Störk
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Corresponding author

Correspondence to Giovanni Palladini.

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Conflict of Interest

Dr. Ihne reports personal fees from Takeda, personal fees from Akcea, other from Janssen, personal fees from ONLUS, grants from IZKF Würzburg, grants from Comprehensive Heart Failure Center (CHFC), personal fees from Pfizer, and personal fees from Alnylam, outside the submitted work

Dr. Morbach reports personal fees from EBR Systems, grants from Bavarian Ministry of Economic Affairs, Regional Development and Energy, Germany, personal fees from Tomtec Imaging Systems, grants from Orion Pharma, personal fees from Alnylam, personal fees from Akcea, and personal fees from Amgen, outside the submitted work.

Dr. Obici reports personal fees from Pfizer, personal fees from Alnylam, and personal fees from Akcea, outside the submitted work

Dr. Störk reports grants from German Ministry for Education and Reserach, grants from European Union, grants from Boehringer Ingelheim, personal fees from Boehringer Ingelheim, personal fees from Bayer, grants from Novartis, other from Novartis, personal fees from Novartis, other from Boehringer Ingelheim, other from Bayer, grants from Bavarian Ministry for Education and Research, and personal fees from Pfizer, outside the submitted work

Dr. Palladini reports personal fees from Janssen-Cilag, personal fees from Takeda, personal fees from Celgene, and personal fees from Prothena, outside the submitted work

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This article does not contain any studies with human or animal subjects performed by any of the authors. All reported studies/experiments with human or animal subjects performed by the authors have been previously published and complied with all applicable ethical standards (including the Helsinki declaration and its amendments, institutional/national research committee standards, and international/national/institutional guidelines).

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This article is part of the Topical Collection on Comorbidities of Heart Failure

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Ihne, S., Morbach, C., Obici, L. et al. Amyloidosis in Heart Failure. Curr Heart Fail Rep 16, 285–303 (2019). https://doi.org/10.1007/s11897-019-00446-x

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