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State-Of-The-Art Radionuclide Imaging in Cardiac Transthyretin Amyloidosis

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State-Of-The-Art Radionuclide Imaging in Cardiac Transthyretin Amyloidosis THEME ARTICLE State-of-the-art radionuclide imaging in cardiac transthyretin amyloidosis Vasvi Singh, MD,a Rodney Falk, MD,b Marcelo F. Di Carli, MD,a Marie Kijewski, PhD,a Claudio Rapezzi, MD,c and Sharmila Dorbala, MDa,b,d a Division of Nuclear Medicine and Department of Radiology, Brigham and Women’s Hospital, Boston, MA b Cardiac Amyloidosis Program, Brigham and Women’s Hospital, Boston, MA c Cardiology, Department of Experimental, Diagnostic and Specialty Medicine, Alma Mater Studiorum, University of Bologna, Bologna, Italy d Cardiac Amyloidosis Program, Cardiovascular Division, Department of Medicine, Heart and Vascular Center, Division of Nuclear Medicine and Department of Radiology, Brigham and Women’s Hospital and Harvard Medical School, Boston, MA Received Nov 14, 2018; accepted Nov 14, 2018 doi:10.1007/s12350-018-01552-4 Cardiac amyloidosis, once considered untreatable, is now gaining well-deserved attention due to advances in imaging and the recent approval of targeted breakthrough therapies. In this paper, we discuss the role of radionuclide imaging in the evaluation and management of patients with the most common form of amyloidosis—cardiac transthyretin amyloidosis (ATTR). We provide a comprehensive summary of the literature interspersed with our institutional experience as appropriate, to deliver our perspective. Key Words: Amyloid heart disease Æ SPECT Æ PET Æ modalities Æ molecular imaging BACKGROUND Cardiac amyloidosis is a disorder in which proteins mis-fold and deposit as amyloid fibrils that infiltrate the Cardiac amyloidosis, once considered untreatable, myocardial extracellular space. Transthyretin protein is now gaining well-deserved attention due to advances (ATTR) derived from the liver or immunoglobulin light in imaging and the recent approval of targeted break- chain proteins (AL), derived from a plasma cell clone, 1,2 through therapies. In this paper, we discuss the role of most commonly affect the heart.3 The former can be due radionuclide imaging in the evaluation and management to variant TTR from TTR gene mutations (ATTRm or of patients with the most common form of amyloido- hereditary amyloidosis) or wild-type TTR deposition sis—cardiac transthyretin amyloidosis (ATTR). We where native TTR proteins mis-fold with aging and provide a comprehensive summary of the literature deposit as fibrils in the heart (ATTRwt, also previously interspersed with our institutional experience as appro- known as senile systemic amyloidosis). With mutant priate, to deliver our perspective. TTR, although over hundred genotypic mutations have Electronic supplementary material The online version of this Reprint requests: Sharmila Dorbala, MD, Cardiac Amyloidosis Pro- article (https://doi.org/10.1007/s12350-018-01552-4) contains sup- gram, Cardiovascular Division, Department of Medicine, Heart and plementary material, which is available to authorized users. Vascular Center, Division of Nuclear Medicine and Department of The authors of this article have provided a PowerPoint file, available Radiology, Brigham and Women’s Hospital and Harvard Medical for download at SpringerLink, which summarises the contents of the School, 75 Francis St, Boston, MA 02115; sdorbala@ paper and is free for re-use at meetings and presentations. Search for bwh.harvard.edu the article DOI on SpringerLink.com. J Nucl Cardiol 2019;26:158–73. Funding Dr. Dorbala is supported by NIH RO1 Grant (RO1 HL 1071-3581/$34.00 130563) and the American Heart Association Grant (AHA 16 CSA Copyright Ó 2018 American Society of Nuclear Cardiology. 2888 0004). Dr. Falk is supported by NIH RO1 Grant (RO1 HL 130563). 158 Journal of Nuclear CardiologyÒ Singh et al 159 Volume 26, Number 1;158–73 State of the art radionuclide imaging been identified in hereditary amyloidosis, variable pen- BONE AVID RADIOTRACERS etrance results in varying degrees of heart involvement.4 Cardiac imaging with bone avid radiotracers has Whereas, at least 50% of primary AL patients5 and been used clinically for over 40 years. Early studies almost all wild-type ATTR patients have cardiac showed high-diagnostic accuracies leading to great involvement,6 but can be challenging to identify. excitement in the field and a profusion of studies. Curiously, some of the later studies reported lower NEED FOR ACCURATE DIAGNOSIS AND TYPING diagnostic accuracies; one paper from 1987 concluded OF CARDIAC AMYLOID FIBRILS that ‘‘when intense uptake of 99mTc-pyrophosphate (PYP) is present amyloidosis is highly likely, but the Diagnosis of cardiac amyloidosis is frequently technique is not sufficiently sensitive.’’14 Astute inves- delayed7 for a number of reasons. Clinical manifesta- tigators, in the early 2000s, recognized greater binding tions are varied, serum cardiac biomarker elevation is avidity of 99mTc-3,3-diphosphono-1,2-propanedicar- non-specific, awareness about the condition is lacking, boxylic acid (DPD) to ATTR (rather than AL). This and until recently, noninvasive techniques for specific new knowledge led to a rebirth of cardiac imaging with diagnosis were not available. Appropriate therapies are bone avid radiotracers for ATTR amyloidosis. delayed in a substantial proportion of the affected individuals. Now, advanced imaging, echocardiography8 and cardiac magnetic resonance (CMR),9 can detect MECHANISM OF BINDING OF BONE AVID cardiac structural and functional changes induced by RADIOTRACERS amyloid deposition. However, they can neither distin- Pyrophosphate (PYP) and closely related bis-phos- guish amyloid from non-amyloid hypertrophic heart phonates (DPD, hydroxymethylene diphosphonate, disease nor ATTR from AL (which is treated very HMDP) labeled with99mTc have been used for imaging differently using chemotherapy). This necessitates a cardiac ATTR amyloidosis (Figure 1). These tracers histological diagnosis. A fat pad biopsy though simple, were used for myocardial infarct imaging in the 1970s has low diagnostic yield in ATTR cardiac amyloidosis and several binding sites have been identified in animal (sensitivity: 15% in wildtype ATTR, 45% in mutant experiments, including microcalcifications, calcium ATTR).10 This is why historically, endomyocardial deposits,15 intracellular calcium PYP,16 or intracellular biopsy has been used to confirm cardiac amyloidosis macromolecules.17 In the context of amyloidosis, the and identify the fibril type. The good news is that data fibril deposits have three major structural components, now supports the value of radionuclide imaging with the precursor protein, heparin sulfate proteoglycan, and bone avid radiotracers to specifically image ATTR a calcium dependent P-component that binds the fibrils cardiac amyloidosis—obviating the need for endomy- together. Pepys et al. suggest that circulating amyloid P ocardial biopsy.11 component, a universal component of all types of Radionuclide imaging plays a critical role in the amyloid, could bind to amyloid fibrils via a calcium diagnosis and identification of ATTR cardiac amyloi- mediated mechanism, also explaining the uptake of dosis. Accurate diagnosis of heart failure from cardiac 99mTc-bone avid tracers.18 More recently, Stats et al.19 amyloidosis has major implications on patient care, as demonstrated that endomyocardial biopsies of ATTR well. Correct identification of hereditary ATTR cardiac hearts had greater density of small microcalcifications amyloidosis has critical and generationally relevant but fewer macrophages when compared with AL hearts. implications for genetic testing and family counseling. Interestingly, even though patients with ATTR cardiac Misidentification of the type of cardiac amyloid can be amyloidosis were older than AL cardiac amyloidosis, life threatening if ATTR patients are misclassified as AL the extent of these microcalcifications did not correlate and erroneously treated with chemotherapy. Conven- with age in either group. It should also be recognised tional heart failure medications, betablockers and that in a few cases of AL cardiac amyloidosis, there angiotensin-converting enzyme inhibitors, are poorly were microcalcification densities comparable with tolerated by patients with amyloidosis and frequently ATTR hearts. This suggests a pathophysiological basis avoided.12 Appropriate use of bone avid radiotracer for reports of borderline positive 99mTc-PYP or 99mTc- imaging can significantly increase the detection of DPD scan in AL patients.20,21 The other clinical scenario patients with cardiac ATTR amyloidosis; these patients, where cardiac microcalcifications might lead to positive will benefit from specific disease modifying therapies scans are myocardial infarcts where uptake is typically targeting the cause of heart failure, TTR, that improve regional and distinct from the diffuse uptake observed in quality of life, heart failure, and prolong life.13 ATTR amyloidosis.15 160 Singh et al Journal of Nuclear CardiologyÒ State of the art radionuclide imaging January/February 2019 99mTc-PYP with 99mTc-DPD or -HMDP imaging in ATTR cardiac amyloidosis is limited. Planar vs SPECT Most experience in imaging protocols for 99mTc- PYP,-DPD, -HMDP is with planar and SPECT imaging routinely (our institutional practice)24 or planar imaging followed by SPECT if planar is positive.14,20,21 Planar imaging alone is limited as myocardial uptake cannot be discerned from blood pool uptake, overlying rib uptake may add counts to the region of the heart (Figure 3), and attenuation correction is not feasible. SPECT overcomes these challenges. Segmental evaluation, and attenuation correction offer the potential advantages
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