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DPD Quantification in Cardiac&Nbsp;Amyloidosis

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DPD Quantification in Cardiac&Nbsp;Amyloidosis JACC: CARDIOVASCULAR IMAGING VOL. 13, NO. 6, 2020 ª 2020 THE AUTHORS. PUBLISHED BY ELSEVIER ON BEHALF OF THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION. THIS IS AN OPEN ACCESS ARTICLE UNDER THE CC BY LICENSE (http://creativecommons.org/licenses/by/4.0/). MINI-FOCUS ISSUE: CARDIAC AMYLOIDOSIS ORIGINAL RESEARCH DPD Quantification in Cardiac Amyloidosis A Novel Imaging Biomarker a,b c a,b Paul R. Scully, MBBS, MRES, Elizabeth Morris, MMATHSPHYS,MSC, Kush P. Patel, MBBS, BSC, a,b c d e Thomas A. Treibel, PHD, Maria Burniston, BSC,PHD, Ernst Klotz, DIPLY PHYS, James D. Newton, MBCHB, MD, e e a,b a Nikant Sabharwal, DM, Andrew Kelion, DM, Charlotte Manisty, PHD, Simon Kennon, MD, a a f a,b Muhiddin Ozkor, MBBS, MD, Michael Mullen, MBBS, MD, Neil Hartman, PHD, Perry M. Elliott, MD, a,g,h i a,b a,j,k Francesca Pugliese, PHD, Philip N. Hawkins, PHD, James C. Moon, MD, Leon J. Menezes, BA, BM BCH ABSTRACT OBJECTIVES To assess whether single-photon emission computed tomography (SPECT/CT) quantification of bone scintigraphy would improve diagnostic accuracy and offer a means of quantifying amyloid burden. BACKGROUND Transthyretin-related cardiac amyloidosis is common and can be diagnosed noninvasively using bone scintigraphy; interpretation, however, relies on planar images. SPECT/CT imaging offers 3-dimensional visualization. METHODS This was a single-center, retrospective analysis of 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) scans reported using the Perugini grading system (0 ¼ negative; 1 to 3 ¼ increasingly positive). Conventional planar quantifi- cation techniques (heart/contralateral lung, and heart/whole-body retention ratios) were performed. Heart, adjacent vertebra, paraspinal muscle and liver peak standardized uptake values (SUVpeak) were recorded from SPECT/CT acquisitions. An SUV retention index was also calculated: (cardiac SUVpeak/vertebral SUVpeak) Â paraspinal muscle SUVpeak. In a subgroup of patients, SPECT/CT quantification was compared with myocardial extracellular volume quantification by CT imaging (ECVCT). RESULTS A total of 100 DPD scans were analyzed (patient age 84 Æ 9 years; 52% male): 40 were Perugini grade 0, 12 were grade 1, 41 were grade 2, and 7 were grade 3. Cardiac SUVpeak increased from grade 0 to grade 2; however, it plateaued between grades 2 and 3 (p < 0.001). Paraspinal muscle SUVpeak increased with grade (p < 0.001), whereas vertebral SUVpeak decreased (p < 0.001). The composite parameter of SUV retention index overcame the plateauing of the cardiac SUVpeak and increased 2 across all grades (p < 0.001). Cardiac SUVpeak correlated well (r ¼ 0.73; p < 0.001) with ECVCT.BoththecardiacSUVpeak and SUV retention index had excellent diagnostic accuracy (area under the curve [AUC]: 0.999). The heart to contralateral lung ratio performed the best of the planar quantification techniques (AUC: 0.987). CONCLUSIONS SPECT/CT quantification in DPD scintigraphy is possible and outperforms planar quantification techniques. Differentiation of Perugini grade 2 or 3 is confounded by soft tissue uptake, which can be overcome by a composite SUV retention index. This index can help in the diagnosis of cardiac amyloidosis and may offer a means of monitoring response to therapy. (J Am Coll Cardiol Img 2020;13:1353–63) © 2020 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/). From the aBarts Heart Centre, St. Bartholomew’s Hospital, London, United Kingdom; bInstitute of Cardiovascular Sciences, University College London, London, United Kingdom; cClinical Physics, St. Bartholomew’s Hospital, London, United Kingdom; dSiemens Healthineers, Forchheim, Germany; eJohn Radcliffe Hospital, Oxford University Hospitals, Oxford, United Kingdom; fNuclear Medicine, Abertawe Bro Morgannwg University HB, Swansea, United Kingdom; gWilliam Harvey Research Institute, Queen Mary University of London, London, United Kingdom; hNIHR Barts Biomedical Research Centre, London, United ISSN 1936-878X https://doi.org/10.1016/j.jcmg.2020.03.020 1354 Scully et al. JACC: CARDIOVASCULAR IMAGING, VOL. 13, NO. 6, 2020 DPD Quantification in Cardiac Amyloidosis JUNE 2020:1353– 63 ABBREVIATIONS myloidosis is a multisystem condi- well prove relevant given the new armamentarium of AND ACRONYMS A tion characterized by the extracellular amyloid-specific therapies in development (8–10). A deposition of abnormally folded pro- semi-quantitative technique was also proposed by AL = amyloidosis, primary fi light-chain amyloidosis tein brils, which result in progressive organ Perugini et al. (6) that involved using the early and late dysfunction (1). Primary light chain (AL) and planar images to calculate heart and whole-body ATTR = transthyretin-related amyloidosis transthyretin-related amyloidosis (ATTR) retention, as well as a heart/whole-body ratio. ATTR-CA = transthyretin- commonly affect the heart; the latter can PYP scintigraphy is an alternative radiotracer used related cardiac amyloidosis either be associated with a TTR gene mutation in the United States for the detection of ATTR-CA, CI = confidence interval (variant ATTR) or not (wild-type ATTR). with imaging currently recommended at 1-h post- CT = computed tomography Previously believed to be rare, more recent injectionwithbothSPECTandplanaracquisitions fi DPD = 99mTc-3,3-diphosphono- research has identi ed cardiac ATTR (ATTR- and optional 3-h SPECT or planar imaging (11). It is 1,2-propanodicarboxylic acid CA) in a significant proportion (14% to 16%) of generally reported using both a visual grading system ECVCT = extracellular volume elderly patients with aortic stenosis (2,3), and a semi-quantitative heart to contralateral lung quantification by computed as well as in 13% of the population with heart (H/CL) ratio from the planar images, with ratios $1.5 tomography failure with preserved ejection fraction (4). at 1 h classified as ATTR-positive (11,12). Furthermore, H/CL ratio = heart to A primary driver of this realization is aH/CLratio$1.6 in patients with ATTR-CA seems to contralateral lung ratio the development of noninvasive diagnostic predict a worse outcome (13). PYP = 99mTc-pyrophosphate techniques, which reduce the need for By comparison, SPECT allows the three- ROI = region of interest endomyocardial biopsy in an often frail and dimensional visualization of radioactivity within the SPECT = single-photon comorbid population. A key technique in body, which can be used to display a standardized emission computed 99m tomography this regard is bone scintigraphy ( Tc-3, uptake value (SUV), a semi-quantitative representa- SUV = standardized uptake 3-diphosphono-1,2-propanodicarboxylic acid tion of the concentration of radiopharmaceutical in value [DPD], 99mTc-pyrophosphate [PYP], and the respective tissues. SPECT quantification has been 99m VOI = volume of interest Tc-hydroxymethylene diphosphonate), used in dementia imaging (14)andtumordosimetry which, coupled with the exclusion of plasma cell in radioimmunotherapy (15). dyscrasia, now offer a noninvasive diagnosis for Cardiac amyloid deposition increases myocardial ATTR-CA (5). extracellular volume (ECV) greater than any other DPD scintigraphy is currently reported by using the nonischemic cardiomyopathy (16), due to the extra- Perugini grading system, which is a visual score of the cellular deposition of the amyloid fibrils. These in- delayed (3-h) planar image, graded from 0 (negative) to creases are detectable using computed tomography 3(stronglypositive)(6). This grading system offers imaging (17), which has been validated against both little prognostic significance (7). Difficulty can also cardiovascular magnetic resonance (18) and invasive ensue in differentiating very subtle cardiac uptake biopsy (19). (i.e., a Perugini grade 1) from a negative scan with some The goal of the current study was to investigate residual blood pool activity, despite the additional use whether SPECT/computed tomography (CT)–derived of single-photon emission computed tomography SUV quantification would improve DPD diagnostic (SPECT) imaging. The clinical importance of this accuracy and offer a means of quantifying amyloid distinction remains to be determined; however, it may burden. Kingdom; iNational Amyloidosis Centre, University College London, London, United Kingdom; jInstitute of Nuclear Medicine, University College London, London, United Kingdom; and the kNIHR University College London Hospitals Biomedical Research Centre, London, United Kingdom. Dr. Scully is supported by a British Heart Foundation Clinical Research Training Fellowship (FS/16/31/32185). Ms. Morris is the recipient of a part time National Institute of Health Research (NIHR) Doctoral Fellowship (NIHR300203); the work presented in this paper does not form part of her fellowship. Dr. Patel is supported by an unrestricted educational grant from Edwards Lifesciences and a British Heart Foundation Clinical research training fellowship grant (FS/19/48/34523). Dr. Treibel is supported by a clinical lecturer grant from the NIHR. Mr. Klotz works for Siemens Healthineers. Dr. Mullen reports consultancy for Abbott Vascular and Edwards Lifesciences; and a research grant from Edwards Lifesciences. Dr. Moon is directly and indirectly supported by the UCLH NIHR Biomedical Research Centre and Biomedical Research Unit at UCLH and Barts, respectively. Dr. Pugliese has received research support from Siemens Healthineers, and this work forms part of the translational research
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