4.7 Article

Diagnosis and treatment of cardiac amyloidosis: a position statement of the ESC Working Group on Myocardial and Pericardial Diseases

Journal

EUROPEAN HEART JOURNAL
Volume 42, Issue 16, Pages 1554-1568

Publisher

OXFORD UNIV PRESS
DOI: 10.1093/eurheartj/ehab072

Keywords

Amyloidosis; Cardiac amyloidosis; Diagnosis; Treatment; AL; TTR; Transthyretin

Funding

  1. Instituto de Salud Carlos III [PI18/0765, PI20/01379]
  2. British Heart Foundation Intermediate Clinical Research Fellowship [FS/18/21/33447]
  3. National Institutes of Health [R01HL139671-01, R21AG058348, K24AG036778]

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Cardiac amyloidosis is a serious infiltrative disease caused by the deposition of amyloid fibrils in the heart, which can be due to genetic variants or acquired conditions. With advances in imaging techniques, non-invasive diagnosis is now possible, showing that cardiac amyloidosis is more common than previously thought. The Working Group on Myocardial and Pericardial Disease has proposed definitions and diagnostic algorithms for cardiac amyloidosis.
Cardiac amyloidosis is a serious and progressive infiltrative disease that is caused by the deposition of amyloid fibrils at the cardiac level. It can be due to rare genetic variants in the hereditary forms or as a consequence of acquired conditions. Thanks to advances in imaging techniques and the possibility of achieving a non-invasive diagnosis, we now know that cardiac amyloidosis is a more frequent disease than traditionally considered. In this position paper the Working Group on Myocardial and Pericardial Disease proposes an invasive and non-invasive definition of cardiac amyloidosis, addresses clinical scenarios and situations to suspect the condition and proposes a diagnostic algorithm to aid diagnosis. Furthermore, we also review how to monitor and treat cardiac amyloidosis, in an attempt to bridge the gap between the latest advances in the field and clinical practice. [GRAPHICS] .

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