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. 2021 Apr 21;42(16):1554-1568.
doi: 10.1093/eurheartj/ehab072.

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

Pablo Garcia-Pavia  1   2   3 , Claudio Rapezzi  4   5 , Yehuda Adler  6 , Michael Arad  7 , Cristina Basso  3   8   9 , Antonio Brucato  10 , Ivana Burazor  11 , Alida L P Caforio  3   12 , Thibaud Damy  3   13 , Urs Eriksson  14 , Marianna Fontana  15 , Julian D Gillmore  15 , Esther Gonzalez-Lopez  1   3 , Martha Grogan  16 , Stephane Heymans  17   18   19 , Massimo Imazio  20 , Ingrid Kindermann  21 , Arnt V Kristen  22   23 , Mathew S Maurer  24 , Giampaolo Merlini  25   26 , Antonis Pantazis  27 , Sabine Pankuweit  28 , Angelos G Rigopoulos  29 , Ales Linhart  30
Affiliations

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

Pablo Garcia-Pavia et al. Eur Heart J. .

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.

Keywords: AL; Amyloidosis; Cardiac amyloidosis; Diagnosis; TTR; Transthyretin; Treatment.

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Figures

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Cardiac amyloidosis
Figure 1
Figure 1
Invasive and non-invasive diagnosis of cardiac amyloidosis. ATTR, transthyretin amyloidosis; CMR, cardiac magnetic resonance; SPIE, serum protein electrophoresis with immunofixation; UPIE, urine protein electrophoresis with immunofixation.
Figure 2
Figure 2
Cardiac uptake grading in bisphosphonate scintigraphy. Grade 0: absence of tracer myocardial uptake and normal bone uptake; Grade 1: myocardial uptake in a lower degree than at bone level; Grade 2: similar myocardial and bone uptake; and Grade 3: myocardial uptake greater than bone with reduced/absent bone uptake.
Figure 3
Figure 3
Screening for cardiac amyloidosis. AV, atrio-ventricular; ECG, electrocardiogram; ECV, extracellular volume; LGE, late gadolinium enhancement.
Figure 4
Figure 4
Diagnostic algorithm for cardiac amyloidosis. AL, light-chain amyloidosis;ATTR, transthyretin amyloidosis; ATTRv, hereditary transthyretin amyloidosis; ATTRwt, wild-type transthyretin amyloidosis; CMR, cardiac magnetic resonance; ECG, electrocardiogram; SPECT, single photon emission computed tomography; TTR, transthyretin.
Figure 5
Figure 5
Treatment of cardiac complications and comorbidities in cardiac amyloidosis. AA, antiarrhythmic; ACEI, angiotensin-converting enzyme inhibitor; AF, atrial fibrillation; ARB, angiotensin receptor blocker; AS, aortic stenosis; ATTRwt, wild-type transthyretin amyloidosis; AV, atrio-ventricular; CRT, cardiac resynchronization therapy; CV, cardioversion; ICD, implantable cardioverter-defibrillator; LVAD, left ventricular assist device; PPM, permanent pacemaker; SR, sinus rhythm; TAVR, transcatheter aortic valve replacement.
Figure 6
Figure 6
Available and future disease-modifying therapies in transthyretin amyloidosis (ATTR). ATTRv, hereditary transthyretin amyloidosis; TTR, transthyretin.
Figure 7
Figure 7
Proposed therapeutic alternatives in transthyretin amyloidosis patients. ATTRv, hereditary transthyretin amyloidosis; ATTRwt, wild-type transthyretin amyloidosis.
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