Abstract | PURPOSE:
Transthyretin (ATTR) amyloidosis is a rare but serious infiltrative disease associated with a wide spectrum of morphologic and functional cardiac involvement. 99mTc-labelled 3,3-diphosphono-1,2-propanodicarboxylic acid (DPD), initially developed as a bone-seeking radiotracer, is remarkably sensitive for imaging cardiac ATTR amyloid deposits. Our aim was to investigate the feasibility and utility of estimating 99mTc-DPD uptake in myocardial tissue; this has the potential to yield reliable quantitative information on cardiac amyloid burden, which is urgently required to monitor disease progression and response to novel treatments. METHODS: Three methods of quantitation were developed and tested on 74 patients with proven cardiac ATTR amyloidosis who had recently undergone 99mTc-DPD planar whole-body imaging and SPECT-CT. Quantitative results were compared to measurements of extracellular volume fraction (ECV) by cardiac magnetic resonance imaging, a validated technique for measuring amyloid burden. RESULTS: An experienced clinician graded uptake using a widely-used visual scoring system as 1 (n = 15), 2 (n = 39) or 3 (n = 20). Linear correlations between the SPECT and ECV data (p < .001) were demonstrated. None of the methods showed that 99mTc-DPD uptake in the heart was significantly greater in patients with grade-3 uptake than in those with grade-2 uptake. CONCLUSIONS: Quantitation of 99mTc-DPD uptake in cardiac transthyretin amyloid deposits is complex and is hindered by competition for radiotracer with amyloid in skeletal muscle. The latter underlies differences in uptake between grade-2 and grade-3 patients, not cardiac uptake.
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Authors | James C Ross, David F Hutt, Maria Burniston, Joanne Page, Jennifer A Steeden, Julian D Gillmore, Ashutosh D Wechalekar, Philip N Hawkins, Marianna Fontana |
Journal | Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
(Amyloid)
Vol. 25
Issue 3
Pg. 203-210
(Sep 2018)
ISSN: 1744-2818 [Electronic] England |
PMID | 30486686
(Publication Type: Journal Article)
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Chemical References |
- Organotechnetium Compounds
- Prealbumin
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Topics |
- Amyloidosis
(metabolism)
- Cardiomyopathies
(metabolism)
- Humans
- Magnetic Resonance Imaging
(methods)
- Organotechnetium Compounds
(metabolism)
- Prealbumin
(genetics)
- Tomography, Emission-Computed, Single-Photon
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