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

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...

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Published in:	European heart journal Vol. 42; no. 16; p. 1554
Main Authors:	Garcia-Pavia, Pablo, Rapezzi, Claudio, Adler, Yehuda, Arad, Michael, Basso, Cristina, Brucato, Antonio, Burazor, Ivana, Caforio, Alida L P, Damy, Thibaud, Eriksson, Urs, Fontana, Marianna, Gillmore, Julian D, Gonzalez-Lopez, Esther, Grogan, Martha, Heymans, Stephane, Imazio, Massimo, Kindermann, Ingrid, Kristen, Arnt V, Maurer, Mathew S, Merlini, Giampaolo, Pantazis, Antonis, Pankuweit, Sabine, Rigopoulos, Angelos G, Linhart, Ales
Format:	Journal Article
Language:	English
Published:	England 21.04.2021
Subjects:	Amyloidosis - diagnosis Amyloidosis - therapy Cardiomyopathies - diagnosis Cardiomyopathies - therapy Heart Heart Diseases - diagnosis Heart Diseases - therapy Humans Myocardium TTR Treatment Cardiac amyloidosis Transthyretin Amyloidosis AL Diagnosis
ISSN:	1522-9645, 1522-9645
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Abstract	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.
AbstractList	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.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. 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.
Author	Eriksson, Urs Heymans, Stephane Basso, Cristina Linhart, Ales Kindermann, Ingrid Pantazis, Antonis Gonzalez-Lopez, Esther Pankuweit, Sabine Rapezzi, Claudio Gillmore, Julian D Imazio, Massimo Rigopoulos, Angelos G Arad, Michael Brucato, Antonio Adler, Yehuda Damy, Thibaud Kristen, Arnt V Burazor, Ivana Caforio, Alida L P Fontana, Marianna Maurer, Mathew S Merlini, Giampaolo Garcia-Pavia, Pablo Grogan, Martha
Author_xml	– sequence: 1 givenname: Pablo surname: Garcia-Pavia fullname: Garcia-Pavia, Pablo organization: European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart – sequence: 2 givenname: Claudio surname: Rapezzi fullname: Rapezzi, Claudio organization: Maria Cecilia Hospital, GVM Care & Research, Cotignola, Italy – sequence: 3 givenname: Yehuda surname: Adler fullname: Adler, Yehuda organization: Leviev Heart Centre, Chaim Sheba Medical Centre (affiliated to Tel Aviv University), Israel – sequence: 4 givenname: Michael surname: Arad fullname: Arad, Michael organization: Heart Failure Institute, Leviev Heart Centre, Sheba Hospital and Sackler School of Medicine, Tel Aviv University, Israel – sequence: 5 givenname: Cristina surname: Basso fullname: Basso, Cristina organization: Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padua, Padua, Italy – sequence: 6 givenname: Antonio surname: Brucato fullname: Brucato, Antonio organization: Dipartimento di Scienze Biomediche e Cliniche, Università degli Studi d Milano, Ospedale Fatebenefratelli, Italy – sequence: 7 givenname: Ivana surname: Burazor fullname: Burazor, Ivana organization: Belgrade University School of Medicine, Cardiology, Institute for Rehabilitation, Belgrade, Serbia – sequence: 8 givenname: Alida L P surname: Caforio fullname: Caforio, Alida L P organization: Cardiology, Department of Cardiac Thoracic Vascular Sciences and Public Health, University of Padova, Padova, Italy – sequence: 9 givenname: Thibaud surname: Damy fullname: Damy, Thibaud organization: French Referral Centre for Cardiac Amyloidosis, Amyloidosis Mondor Network, GRC Amyloid Research Institute, CHU Henri Mondor, Créteil, France – sequence: 10 givenname: Urs surname: Eriksson fullname: Eriksson, Urs organization: GZO-Zurich Regional Health Centre, Wetzikon & Cardioimmunology, Centre for Molecular Cardiology, University of Zurich, Switzerland – sequence: 11 givenname: Marianna surname: Fontana fullname: Fontana, Marianna organization: National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Hospital, London, UK – sequence: 12 givenname: Julian D surname: Gillmore fullname: Gillmore, Julian D organization: National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Hospital, London, UK – sequence: 13 givenname: Esther surname: Gonzalez-Lopez fullname: Gonzalez-Lopez, Esther organization: European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart – sequence: 14 givenname: Martha surname: Grogan fullname: Grogan, Martha organization: Cardiac Amyloid Clinic, Division of Circulatory Failure, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA – sequence: 15 givenname: Stephane surname: Heymans fullname: Heymans, Stephane organization: ICIN-Netherlands Heart Institute, Holland Heart House, Utrecht, Netherlands – sequence: 16 givenname: Massimo surname: Imazio fullname: Imazio, Massimo organization: Cardiovascular and Thoracic Department, University Cardiology, AOU Città della Salute e della Scienza di Torino, Torino, Italy – sequence: 17 givenname: Ingrid surname: Kindermann fullname: Kindermann, Ingrid organization: Department of Internal Medicine III (Cardiology, Angiology and Intensive Care), Saarland University Medical Centre, Saarland University, Homburg/Saar, Germany – sequence: 18 givenname: Arnt V surname: Kristen fullname: Kristen, Arnt V organization: Cardiovascular Centre Darmstadt, Heidelberg, Germany – sequence: 19 givenname: Mathew S surname: Maurer fullname: Maurer, Mathew S organization: Cardiac Amyloidosis Program, Centre for Advanced Cardiac Care, Columbia University Irving Medical Centre, New York Presbyterian Hospital, New York, NY, USA – sequence: 20 givenname: Giampaolo surname: Merlini fullname: Merlini, Giampaolo organization: Department of Molecular Medicine, University of Pavia, Italy – sequence: 21 givenname: Antonis surname: Pantazis fullname: Pantazis, Antonis organization: Cardiomyopathy Service, Royal Brompton Hospital, London, UK – sequence: 22 givenname: Sabine surname: Pankuweit fullname: Pankuweit, Sabine organization: Department Of Cardiology, Philipps-University Marburg, Marburg, Germany – sequence: 23 givenname: Angelos G surname: Rigopoulos fullname: Rigopoulos, Angelos G organization: Mid-German Heart Centre, Department of Internal Medicine III, Division of Cardiology, Angiology and Intensive Medical Care, University Hospital Halle, Martin-Luther-University Halle-Wittenberg, Halle (Saale), Germany – sequence: 24 givenname: Ales surname: Linhart fullname: Linhart, Ales organization: 2nd Department of Medicine, Department of Cardiovascular Medicine, First Faculty of Medicine, Charles University, General University Hospital, Prague, Czech Republic
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ContentType	Journal Article
Copyright	European Society of Cardiology 2021 This article has been co-published with permission in European Heart Journal (published by Oxford University Press on behalf of European Society of Cardiology) and European Journal of Heart Failure (published by John Wiley & Sons Ltd on behalf of European Society of Cardiology) These articles are identical except for minor stylistic and spelling differences in keeping with each journal’s style. Either citation can be used when citing this article.
Copyright_xml	– notice: European Society of Cardiology 2021 This article has been co-published with permission in European Heart Journal (published by Oxford University Press on behalf of European Society of Cardiology) and European Journal of Heart Failure (published by John Wiley & Sons Ltd on behalf of European Society of Cardiology) These articles are identical except for minor stylistic and spelling differences in keeping with each journal’s style. Either citation can be used when citing this article.
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DOI	10.1093/eurheartj/ehab072
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License	European Society of Cardiology 2021 This article has been co-published with permission in European Heart Journal (published by Oxford University Press on behalf of European Society of Cardiology) and European Journal of Heart Failure (published by John Wiley & Sons Ltd on behalf of European Society of Cardiology) These articles are identical except for minor stylistic and spelling differences in keeping with each journal’s style. Either citation can be used when citing this article.
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Snippet	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...
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SubjectTerms	Amyloidosis - diagnosis Amyloidosis - therapy Cardiomyopathies - diagnosis Cardiomyopathies - therapy Heart Heart Diseases - diagnosis Heart Diseases - therapy Humans Myocardium
Title	Diagnosis and treatment of cardiac amyloidosis: a position statement of the ESC Working Group on Myocardial and Pericardial Diseases
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