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PIIS1071916419313971.Pdf Journal of Cardiac Failure Vol. 25 No. 11 2019 ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI Expert Consensus Recommendations for Multimodality Imaging in Cardiac Amyloidosis: Part 1 of 2—Evidence Base and Standardized Methods of Imaging SHARMILA DORBALA, MD, MPH, FASNC,1 YUKIO ANDO, MD, PhD,2 SABAHAT BOKHARI, MD,3 ANGELA DISPENZIERI, MD,4 RODNEY H. FALK, MD,1 VICTOR A. FERRARI, MD,5 MARIANNA FONTANA, PhD,6 OLIVIER GHEYSENS, MD, PhD,7 JULIAN D. GILLMORE, MD, PhD,6 ANDOR W.J.M. GLAUDEMANS, MD, PhD,8 MAZEN A. HANNA, MDv,9 BOUKE P.C. HAZENBERG, MD, PhD,10 ARNT V. KRISTEN, MD,11 RAYMOND Y. KWONG, MD, MPH,1 MATHEW S. MAURER, MD,3 GIAMPAOLO MERLINI, MD,12,13 EDWARD J. MILLER, MD, PhD,14 JAMES C. MOON, MD,6 VENKATESH L. MURTHY, MD, PhD,15 C. CRISTINA QUARTA, MD, PhD,6 CLAUDIO RAPEZZI, MD,16 FREDERICK L. RUBERG, MD,17 SANJIV J. SHAH, MD,18 RIEMER H.J.A. SLART, MD,8 HEIN J. VERBERNE, MD, PhD,19 AND JAMIESON M. BOURQUE, MD, MHS, FASNC20 Boston, Massachusetts; Kumamoto, Japan; New York, New York; Rochester, Minnesota; Philadelphia, Pennsylvania; London, United Kingdom; Leuven, Belgium; Groningen, and Amsterdam, The Netherlands; Cleveland, Ohio; Heidelberg, Germany; Pavia, and Bologna, Italy; New Haven, Connecticut; Ann Arbor, Michigan; Chicago, Illinois; and Charlottesville, Virginia Preamble an abundance of diagnostic imaging options, cardiac amy- Cardiac amyloidosis is a form of restrictive infiltrative loidosis remains largely underrecognized or delayed in cardiomyopathy that confers significant mortality. Because diagnosis.4 Although advanced imaging options for nonin- of the relative rarity of cardiac amyloidosis, clinical and vasive evaluation have substantially expanded, the evidence diagnostic expertise in the recognition and evaluation of is predominately confined to single-center small studies or individuals with suspected amyloidosis is mostly limited to limited multicenter larger experiences, and there continues a few expert centers. Electrocardiography, echocardiogra- to be no clear consensus on standardized imaging pathways phy, and radionuclide imaging have been used for the eval- in cardiac amyloidosis. This lack of guidance is particularly À uation of cardiac amyloidosis for over 40 years.1 3 problematic given that there are numerous emerging thera- Although cardiovascular magnetic resonance (CMR) has peutic options for this morbid disease, increasing the impor- also been in clinical practice for several decades, it was not tance of accurate recognition at earlier stages. Imaging applied to cardiac amyloidosis until the late 1990s. Despite provides noninvasive tools for follow-up of disease From the 1Cardiac Amyloidosis Program, Cardiovascular Imaging Program, Departments of Radiology and Medicine, Harvard Medical School, Brigham and Women’s Hospital, Boston, Massachusetts; 2Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan; 3Columbia University Medical Center/New York Presbyterian Hospital, Columbia University, New York, New York; 4Division of Hematology, Division of Cardiovascular Diseases, and Department of Radiology, Division of Nuclear Medicine, Department of Medicine, Mayo Clinic, Rochester, Minnesota; 5Perel- man School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; 6Division of Medicine, National Amyloidosis Centre, University College London, London, United Kingdom; 7Nuclear Medicine and Molecular Imaging, University Hospitals Leuven, Leuven, Belgium; 8Department of Nuclear Med- icine and Molecular Imaging, Medical Imaging Center, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands; 9Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, Ohio; 10Department of Rheumatology & Clinical Immunology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands; 11Department of Cardiology, University of Heidelberg, Heidelberg, Germany; 12Amyloidosis Research and Treatment Center, Foundation Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy; 13Depart- ment of Molecular Medicine, University of Pavia, Pavia, Italy; 14Cardiovascular Medicine, Yale University School of Medicine, New Haven, Connecticut; 15Frankel Cardiovascular Center, Michigan Medicine, Ann Arbor, Michigan; 16Cardiology Unit, Department of Experimental, Diagnostic and Specialty Medicine, Alma Mater-University of Bologna, Bologna, Italy; 17Amyloidosis Center and Section of Cardiovascular Medicine, Department of Medicine, Bos- ton University School of Medicine, Boston Medical Center, Boston, Massachusetts; 18Feinberg School of Medicine, Northwestern University, Chicago, Illi- nois; 19Department of Radiology and Nuclear Medicine, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands and 20Departments of Medicine and Radiology, Cardiovascular Imaging Center, University of Virginia, Charlottesville, Virginia. Reprint requests: Sharmila Dorbala, MD, MPH, FASNC (Chair), Cardiac Amyloidosis Program, Cardiovascular Imaging Program, Departments of Radiol- ogy and Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston MA. E-mail: [email protected] This article is being jointly published in the Journal of Nuclear Cardiology, the Journal of Cardiac Failure, and Circulation: Cardiovascular Imaging. Part 2 - Diagnostic Criteria and Appropriate Utilization is available at https://doi.org/10.1007/s12350-019-01761-5. This document was approved for publication by the governing body of the American Society of Nuclear Cardiology (ASNC) and was endorsed by the American College of Cardiology (ACC), the American Heart Association (AHA), American Society of Echocardiography (ASE), the European Association of Nuclear Medicine (EANM), the Heart Failure Society of America (HFSA), the International Society of Amyloidosis (ISA), the Society for Cardiovascular Magnetic Resonance (SCMR), and the Society of Nuclear Medicine and Molecular Imaging (SNMMI). See page e23 for disclosure information. 1071-9164/$ - see front matter © 2019 Published by Elsevier Inc. https://doi.org/10.1016/j.cardfail.2019.08.001 e1 e2 Journal of Cardiac Failure Vol. 25 No. 11 November 2019 remission/progression complementing clinical evaluation. Overview of Cardiac Amyloidosis Additional areas not defined include appropriate clinical Cardiac amyloidosis is a cardiomyopathy that results in indications for imaging, optimal imaging utilization by clin- restrictive physiology from the myocardial accumulation of ical presentation, accepted imaging methods, accurate misfolded protein deposits, termed amyloid fibrils, causing image interpretation, and comprehensive and clear report- a clinically diverse spectrum of systemic diseases. Most ing. Prospective randomized clinical trial data for the diag- cases of cardiac amyloidosis result from 2 protein precur- nosis of amyloidosis and for imaging-based strategies for sors: amyloid immunoglobulin light chain (AL), in which treatment are not available. A consensus of expert opinion the misfolded protein is a monoclonal immunoglobulin light is greatly needed to guide the appropriate clinical utilization chain typically produced by bone marrow plasma cells, and of imaging in cardiac amyloidosis. amyloid transthyretin (ATTR) amyloidosis, in which the misfolded protein is transthyretin (TTR), a serum transport Introduction protein for thyroid hormone and retinol that is synthesized The American Society of Nuclear Cardiology (ASNC) primarily by the liver.3 ATTR amyloidosis is further sub- has assembled a writing group with expertise in cardiovas- typed by the sequence of the TTR protein into wild-type cular imaging and amyloidosis, with representatives from (ATTRwt) or hereditary (ATTRv), the latter resulting from the American College of Cardiology (ACC), the American genetic variants in the TTR gene.5,6 Cardiac involvement in Heart Association (AHA), the American Society of Echo- systemic AL amyloidosis is common (up to 75%, depending cardiography (ASE), the European Association of Nuclear on diagnostic criteria),7 and in the case of ATTRwt amy- Medicine (EANM), the Heart Failure Society of America loidosis, is the dominant clinical feature seen in all cases. (HFSA), the International Society of Amyloidosis (ISA), The different types of cardiac amyloidosis display signifi- the Society of Cardiovascular Magnetic Resonance imag- cant heterogeneity in clinical course, prognosis, and treat- ing (SCMR), and the Society of Nuclear Medicine and ment approach.8 AL amyloidosis is characterized by a Molecular Imaging (SNMMI). This writing group has rapidly progressive clinical course, and if untreated, the developed a joint expert consensus document on imaging median survival is less than 6 months. ATTRv amyloidosis cardiac amyloidosis, divided into 2 parts. Part 1 has the follows a varied clinical course depending upon the specific following aims: mutation inherited with either cardiomyopathy and/or sen- sory/autonomic polyneuropathy.9 Furthermore, ATTR amy- loidosis (both wild-type and hereditary) is characterized by 1) Perform and document a comprehensive review of exist- an age-dependent penetrance, with the clinical phenotype ing evidence on the utility of echocardiography, CMR, developing as age advances. and radionuclide imaging in screening, diagnosis, and The diagnosis of cardiac amyloidosis remains challeng- management of cardiac amyloidosis. ing owing to a number of factors, which include the relative 2) Define standardized technical protocols for the acqui- rarity of
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