Ando, Y

Bokhari, S

Bourque, JM

Dispenzieri, A

Dorbala, S

Falk, RH

Ferrari, VA

Fontana, M

Gheysens, O

Gillmore, JD

Glaudemans, AWJM

Hanna, MA

Hazenberg, BPC

Kristen, AV

Kwong, RY

Maurer, MS

Merlini, G

Miller, EJ

Moon, JC

Murthy, VL

Quarta, CC

Rapezzi, C

Ruberg, FL

Shah, SJ

Slart, RHJA

Verberne, HJ

English

UCL Discovery

 Journal Pre-proofnAddendum to ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMIexpert consensus recommendations for multimodality imaging incardiac amyloidosis: Part 1 of 2—evidence base and standardizedmethods of imagingSharmila Dorbala MD, MPH, FASNC , Yukio Ando MD, PhD ,Sabahat Bokhari MD , Angela Dispenzieri MD ,Rodney H. Falk MD , Victor A. Ferrari MD ,Marianna Fontana PhD , Olivier Gheysens MD, PhD ,Julian D. Gillmore MD,PhD , Andor W.J.M. Glaudemans MD, PhD ,Mazen A. Hanna MD , Bouke P.C. Hazenberg MD, PhD ,Arnt V. Kristen MD , Raymond Y. Kwong MD, MPH ,Mathew S. Maurer MD , Giampaolo Merlini MD ,Edward J. Miller MD, PhD , James C. Moon MD ,Venkatesh L. Murthy MD,PhD , C. Cristina Quarta MD, PhD ,Claudio Rapezzi MD , Frederick L. Ruberg MD ,Sanjiv J. Shah MD , Riemer H.J.A. Slart MD ,Hein J. Verberne MD, PhD ,Jamieson M. Bourque MD, MHS, FASNCPII: S1071-9164(19)31397-1DOI: https://doi.org/10.1016/j.cardfail.2021.06.012Reference: YJCAF 4816To appear in: Journal of Cardiac FailurePlease cite this article as: Sharmila Dorbala MD, MPH, FASNC , Yukio Ando MD, PhD ,Sabahat Bokhari MD , Angela Dispenzieri MD , Rodney H. Falk MD , Victor A. Ferrari MD ,Marianna Fontana PhD , Olivier Gheysens MD, PhD , Julian D. Gillmore MD,PhD ,Andor W.J.M. Glaudemans MD, PhD , Mazen A. Hanna MD , Bouke P.C. Hazenberg MD, PhD ,Arnt V. Kristen MD , Raymond Y. Kwong MD, MPH , Mathew S. Maurer MD ,Giampaolo Merlini MD , Edward J. Miller MD, PhD , James C. Moon MD , Venkatesh L. Murthy MD,PhD ,C. Cristina Quarta MD, PhD , Claudio Rapezzi MD , Frederick L. Ruberg MD , Sanjiv J. Shah MD ,Riemer H.J.A. Slart MD , Hein J. Verberne MD, PhD , Jamieson M. Bourque MD, MHS, FASNC ,nAddendum to ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI expert consensus rec-ommendations for multimodality imaging in cardiac amyloidosis: Part 1 of 2—evidencebase and standardized methods of imaging, Journal of Cardiac Failure (2021), doi:https://doi.org/10.1016/j.cardfail.2021.06.012This is a PDF file of an article that has undergone enhancements after acceptance, such as the additionof a cover page and metadata, and formatting for readability, but it is not yet the definitive version ofrecord. This version will undergo additional copyediting, typesetting and review before it is publishedin its final form, but we are providing this version to give early visibility of the article. Please note that,during the production process, errors may be discovered which could affect the content, and all legaldisclaimers that apply to the journal pertain.© 2021 Published by Elsevier Inc.Addendum to 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,a Yukio Ando, MD, PhD,b Sabahat Bokhari, MD,c Angela Dispenzieri, MD,d Rodney H. Falk, MD,a Victor A. Ferrari, MD,e Marianna Fontana, PhD,f Olivier Gheysens, MD, PhD,g Julian D. Gillmore, MD,PhD,f Andor W. J. M. Glaudemans, MD, PhD,h Mazen A. Hanna, MD,i Bouke P. C. Hazenberg, MD, PhD,j Arnt V. Kristen, MD,k Raymond Y. Kwong, MD, MPH,a Mathew S. Maurer, MD,c Giampaolo Merlini, MD,l,mEdward J. Miller, MD, PhD,n James C. Moon, MD,f Venkatesh L. Murthy, MD,PhD,o C. Cristina Quarta, MD, PhD,f Claudio Rapezzi, MD,p Frederick L. Ruberg, MD,q Sanjiv J. Shah, MD,r Riemer H. J. A. Slart, MD,h Hein J. Verberne, MD, PhD,s and Jamieson M. Bourque, MD, MHS, FASNCt a Cardiac Amyloidosis Program, Cardiovascular Imaging Programs, Departments of Medicineand Radiology, Harvard Medical School, Brigham and Women’s Hospital, Boston, MA b  Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan c Columbia University Medical Center, New York Presbyterian Hospital, Columbia University, New York, NY d  Division of Hematology, Division of Cardiovascular Diseases, Department of Radiology, Division of Nuclear Medicine, Department of Medicine, Mayo Clinic, Rochester, MN e Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA f  Division of Medicine, National Amyloidosis Centre, University College London, London, UK g University Hospitals Leuven, Nuclear Medicine and Molecular Imaging, Leuven, Belgium h  Department of Nuclear Medicine and Molecular Imaging, Medical Imaging Center, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands i  Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, OH j  Department of Rheumatology & Clinical Immunology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands k  Department of Cardiology, University of Heidelberg, Heidelberg, Germany l   Foundation Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Amyloidosis Research and Treatment Center, Pavia, Italy m Department of Molecular Medicine, University of Pavia, Pavia, Italy n  Cardiovascular Medicine, Yale University School of Medicine, New Haven, CT o Frankel Cardiovascular Center, Michigan Medicine, Ann Arbor, MI          p Cardiology Unit, Department of Experimental, Diagnostic and Specialty Medicine, Alma Mater-University of Bologna, Bologna, Italy q Department of Medicine, Amyloidosis Center and Section of Cardiovascular Medicine, Boston Medical Center, Boston University School of Medicine, Boston, MA r  Feinberg School of Medicine, Northwestern University, Chicago, IL s  Department of Radiology and Nuclear Medicine, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands t   Departments of Medicine and Radiology, Cardiovascular Imaging Center, University of Virginia, Charlottesville, VA Reprint requests: Jamieson M. Bourque, MD, MHS, FASNC (Co- Chair), Departments of Medicine and Radiology, Cardiovascular Imaging Center,  University  of Virginia, Charlottesville, VA; JMB8T@hscmail.mcc.virginia.edu  THE NEED FOR AN ADDENDUM There are two primary reasons for an addendum. The first is that the document reviewer list is being updated to include Dr. Richard Cheng and Dr. Roy John, who have critically reviewed the document, but were inadvertently not listed as reviewers. In addition, since the publication of this document and introduction of approved therapies for transthyretin cardiac amyloidosis (ATTR-CA), the clinical use of bone tracer cardiac scintigraphy has been extended to populations with lower prevalence of ATTR-CA. Numerous observations have raised concerns about (1) incorrect diagnosis of ATTR-CA based on 99mTc-pyrophosphate (PYP) planar imaging and heart-to-contralateral lung (H/CL) ratio without confirmation of diffuse myocardial uptake on SPECT imaging at some sites; (2) excess blood pool activity on the |-hour planar and SPECT images being 2 interpreted as positive scans; and (3) missed diagnosis of light chain (AL) amyloidosis, as serum-free light chain studies and serum and urine immunofixation electrophoresis studies may not be recommended in the °°MT ¢-PY P/-3,3-diphosphono- 1 ,2-propanodicarboxylic acid/hydroxymethylene diphosphonate (99mTc-PYP/DPD/HMDP) report. Incorrect diagnosis leads to inappropriate therapy and worse patient outcomes. SPECT and planar imaging performed at 3 hour maximize specificity. Additionally, technical parameters have been updated. Accordingly, we are issuing this addendum to clarify the protocols, interpretation, and reporting of 2 MTc-PYP imaging: 1. Acquisition (Table 4): a. The time between injection of 99mTc-PYP and scan is revised: 2- or 3-hour imaging 1s recommended, and |-hour imaging is optional (Table 4). If excess blood pool activity is noted, 3-hour imaging is recommended. The timing between injection and scanning is now consistent for 99mTc-PYP, -DPD, and -HMDP. We recognize some experienced centers that have become proficient at 1-hour scanning; the recommendation for 2- or 3-hour imaging is particularly important for centers starting new 99mTc-PYP programs.          b. SPECT imaging is required in all studies (irrespective of time between injection and scan) to highlight the importance of directly visualizing tracer uptake in the myocardium. c. 1-hour planar-only imaging is not recommended. d. Emerging literature suggests that cadmium zinc telluride (CZT) SPECT can also be used for 99mT¢-PYP/DPD/HMDP imaging.*” 2. Interpretation (Table 5): a. Planar imaging and H/CL ratio alone are insufficient for diagnosis of ATTR cardiac amyloidosis. SPECT imaging is necessary to identify myocardial uptake of ??"Tc-PYP/DPD/HMDP. b. Repeat imaging is recommended at 3 hours if excess blood pool activity is noted. c. The steps in Table 5 clarify that visual grading on planar and SPECT imaging 1s the primary method for diagnosis of ATTR cardiac amyloidosis. d. Recommendations are clarified for ease of interpretation. 3. Reporting (Table 6): a. Diffuse myocardial uptake should be visualized to report a positive scan. b. The criterion for H/CL ratio > 1.5 as strongly positive has been removed (consistent with diagnostic criteria listed in the ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI Expert Consensus Recommendations for Multimodality Imaging in Cardiac Amyloidosis: Part 2 of 2—Diagnostic Criteria and Appropriate Utilization, where H/CL ratio was not listed). c. Conclusions have been clarified. References 1. Sperry BW, Burgett E, Bybee KA, McGhie AI, O’ Keefe JH, Saeed IM, et al. Technetium pyrophosphate nuclear scintigraphy for cardiac amyloidosis: Imaging at | vs 3 hours and planar vs SPECT/CT. J Nucl Cardiol 2020;27(5):1802—7. https://doi.org/10.1007/s12350-020-02 139-8. 2. Masri A, Bukhari S, Ahmad S, Nieves R, Eisele YS, Follansbee W, et al. Efficient 1-hour Technetium-99m pyrophosphate imaging protocol for the diagnosis of Transthyretin Cardiac Amyloidosis. Circ Cardiovasc Imaging 2020;13:e010249. https://doi.org/10.1161/CIRCIMAGING.119.010249. 3. Castano A, Haq M, Narotsky DL, Goldsmith J, Weinberg RL, Morgenstern R, et al. Multicenter study of planar Technetium 99m pyrophosphate cardiac imaging: Predicting survival for patients With ATTR Cardiac Amyloidosis. JAMA Cardiol 2016;1(8):880-9. https://doi.org/10.1001/jamacardio.2016.2839. 4. Tamarappoo B, Otaki Y, Manabe O, Hyun M, Cantu S, Arnson Y, et al. Simultaneous Tc-99m PYP/TI-201 dual-isotope SPECT myocardial imaging in patients with suspected cardiac amyloidosis. J Nucl Cardiol 2020;27:28—37.          5. Manrique A, Dudoignon D, Brun S, N’Ganoa C, Cassol E, Legallois D, et al. Quantification of myocardial (99m)Tc-labeled bisphosphonate uptake with cadmium zinc telluride camera in patients with transthyretin-related cardiac amyloidosis. EJNMMI _ Res 2019;9:117.  Table 4. Recommendations for standardized acquisition of 99mTc-PYP/DPD/HMDP for cardiac amyloidosis. Adapted from Reference (207) Imaging procedures Parameters Recommendation Preparation No specific preparation. No fasting required Required Scan Rest scan Required Dose 99mTc-PYP: 10-20 mCi (370-740 MBq) intravenously Recommended  99mTc-DPD: 10-20 mCi (370-740 MBq) intravenously   99mTc-HMDP: 10-20 mCi (370-740 MBq) intravenously Recommended Time between injection and acquisition:°>?"Tc-PYP/DPD/HMDP 2 or 3h Optional. If excess blood pool activity noted on 1-h images, 3-h imaging is recommended. Time between injection and acquisition:°> 99mTc-PYP only 1h See below regarding image type. General imaging parameters’ Field of view Heart Required  Chest Optional for planar CT attenuation correction Heart Recommended Image type: planar Chest SPECT/CT fusion images helpful to localize tracer uptake to the myocardium  2 or3h Recommended Image type: SPECT Position Heart 1-hour planar-only imaging is not recommended  Supine Required  Upright Required Energy window 140 keV, 15-20% Optional Collimators Low energy, high resolution Required Matrix-Planar 256 x 256 Recommended Matrix-SPECT 128 x 128 (at least 64 by 64 is required) Recommended Pixel size 2.3-6.5 mm Recommended Planar imaging specific parameters’ Number of views* Anterior and lateral Recommended Detector configuration 90° Required Image duration (count based) 750,000 counts Recommended Magnification 1.46 for large field of view systems Recommended SPECT imaging specific parameter† 1.0 for small field of view systems Recommended with goal of achieving recommended pixel          size Angular range/detector configur 180°/90° Recommended  360°/180° Minimum Required ECG gating Off; Non-gated imaging Optional, recommended if large FOV camera is available Number of views/detector 40/32 Recommended Time per stop 20 s/25 5 Recommended Magnification 1.46 (180° angular range) Recommended  1.0 (360° angular range) Recommended ECG electrocardiogram, PYP pyrophosphate *Anterior and lateral views are obtained at the same time; lateral planar views or SPECT imaging may help separate sternal from myocardial uptake †Parameters for Nal SPECT scanners            Table 5. Recommendations for interpretation of 99mTc-PYP/DPD/HMDP for cardiac amyloidosis. Adapted from Reference (207) Step 1: Visual interpretation Evaluate planar and SPECT images to confirm diffuse radiotracer uptake in the myocardium Differentiate myocardial radiotracer uptake from residual blood pool activity, focal myocardial infarct, and overlapping bone (e.g., from rib hot spots from fractures) on SPECT images. If excess blood pool activity is noted, recommend repeat SPECT imaging at 3 h If myocardial tracer uptake is visually present on SPECT, proceed to step 2, semi-quantitative visual grading. If no myocardial tracer uptake is present on SPECT, the visual grade is 0 Step 2: Semi-quantitative visual grading to diagnose ATTR cardiac amyloidosis Examine planar and SPECT images for relative tracer uptake in the myocardium relative to ribs and grade using the following scale: Grade 0 No myocardial uptake and normal bone uptake Grade 1 Myocardial uptake less than rib uptake Grade 2 Myocardial uptake equal to rib uptake Grade 3 Myocardial uptake greater than rib uptake with mild/absent rib uptake Step 3: Heart/contralateral lung uptake ratio assessment (when applicable) A circular ROI should be drawn over the heart on the anterior planar images with care to avoid sternal overlap and with size adjusted to maximize coverage of the heart without inclusion of adjacent lung. This ROI (same size) should be mirrored over the contralateral chest without inclusion of the right ventricle, to adjust for background and rib uptake (see Fig. 6*). The heart and contralateral ROIs should be drawn above the diaphragm An H/CL ratio is calculated as the fraction of heart ROI mean counts to contralateral lung ROI mean counts. H/CL ratios of C 1.5 at 1 h can accurately identify ATTR cardiac amyloidosis if myocardial PYP uptake is visually confirmed on SPECT and systemic AL amyloidosis is excluded (114). An H/CL ratio of C 1.3 at 3 h can identify ATTR cardiac amyloidosis NOTE: Diagnosis of ATTR cardiac amyloidosis cannot be made solely based on H/CL ratio alone with PYP. H/CL ratio is not recommended if there is absence of myocardial uptake on SPECT. Additionally, if the visual grade is 2 or 3, diagnosis is confirmed and H/CL ratio assessment is not necessary. H/CL ratio is typically concordant with visual grade. If discordant or the visual grade is equivocal, H/CL ratio may be helpful to classify equivocal visual grade 1 versus 2 as positive or negative See Fig. 7.* Grade 2 or Grade 3 uptake is consistent with ATTR cardiac amyloidosis if a monoclonal plasma cell dyscrasia is excluded, as this degree of uptake can be seen in [ 20% of patients with AL cardiac amyloidosis.(3) Grade 0 and Grade 1 uptake may be observed in AL cardiac amyloidosis and warrants further evaluation to exclude AL amyloidosis.(3) The writing group would like to emphasize the importance of excluding a monoclonal process with serum/urine immunofixation and a serum-free light-chains assay in all patients with suspected amyloidosis Of note: 99mTc-PYP/DPD/HMDP uptake could be seen in other causes of myocardial injury, including pericarditis, myocardial infarction (regional uptake), and chemotherapy or drug associated myocardial toxicity *Fig. 6 and 7 refer to figures in the original document AL amyloid light chain, ATTR amyloid transthyretin, H/CL heart/contralateral lung, ROI region of interest          Table 6. Recommendations for standardized reporting of 99mTc-PYP/DPD/HMDP imaging for cardiac amyloidosis. Adapted from Reference (207) Parameters Elements Demographics Patient name, age, sex, reason for the test, date of study, prior imaging procedures, biopsy results if available (required) Methods Imaging technique, radiotracer dose and mode of administration, interval between injection and scan, scan technique (planar and SPECT) (required) Findings Image quality Visual scan interpretation (required) Semi-quantitative interpretation in relation to rib uptake (required) Quantitative findings H/CL lung ratio (optional; recommended for positive scans) Ancillary findings Whole-body imaging if planar whole-body images are acquired (optional) Interpret CT for attenuation correction if SPECT/CT scanners are used (recommended) Conclusions 1. An overall interpretation of the findings into categories of (1) not suggestive of ATTR cardiac amyloidosis; (2) strongly suggestive of ATTR cardiac amyloidosis; or (3) equivocal for ATTR cardiac amyloidosis after exclusion of a systemic plasma cell dyscrasia (required) (a) Not suggestive: A semi-quantitative visual Grade of O (b) Equivocal: If diffuse myocardial uptake of 99mTc-PYP/DPD/HMDP is visually confirmed and the semi-quantitative visual grade is 1 or there is interpretive uncertainty of grade 1 versus grade 2 on visual grading (c) Strongly suggestive: If diffuse myocardial uptake of 99mTc-PYP/DPD/HMDP is visually confirmed, a semi-quantitative visual grade of 2 or 3 2. Statement that evaluation for AL amyloidosis by serum FLCs, serum, and urine immunofixation is recommended in all patients undergoing 99mTc-PYP/DPD/HMDP scans for cardiac amyloidosis (required) 3. Statement that results should be interpreted in the context of prior evaluation and referral toa hematologist or amyloidosis expert is recommended if either: (a) Recommended echo/CMR is strongly suggestive of cardiac amyloidosis and 99mTc- PYP/DPD/HMDP is not suggestive or equivocal and/or (b) FLCs are abnormal or equivocal (recommended)            

Addendum to 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

https://discovery.ucl.ac.uk/10131091/1/10.1016%20j.cardfail.2021.06.012.pdf

Addendum to 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

Abstract

Similar works

Full text

Available Versions

UCL Discovery