Cardiac [99mTc]Tc-hydroxydiphosphonate uptake on bone scintigraphy in patients with hereditary transthyretin amyloidosis: an early follow-up marker?

H. S.A. Tingen*, A. Tubben, J. Bijzet, M. P. van den Berg, P. van der Meer, E. J. Houwerzijl, F. L.H. Muntinghe, P. A. van der Zwaag, A. W.J.M. Glaudemans, M. I.F.J. Oerlemans, C. Knackstedt, M. Michels, A. Hirsch, B. P.C. Hazenberg, R. H.J.A. Slart, H. L.A. Nienhuis

*Corresponding author for this work

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Abstract

Purpose: There is a need for early quantitative markers of potential treatment response in patients with hereditary transthyretin (ATTRv) amyloidosis to guide therapy. This study aims to evaluate changes in cardiac tracer uptake on bone scintigraphy in ATTRv amyloidosis patients on different treatments. Methods: In this retrospective cohort study, outcomes of 20 patients treated with the transthyretin (TTR) gene silencer patisiran were compared to 12 patients treated with a TTR-stabilizer. Changes in NYHA class, cardiac biomarkers in serum, wall thickness, and diastolic parameters on echocardiography and NYHA class during treatment were evaluated. Results: Median heart/whole-body (H/WB) ratio on bone scintigraphy decreased from 4.84 [4.00 to 5.31] to 4.16 [3.66 to 4.81] (p <.001) in patients treated with patisiran for 29 [15–34] months. No changes in the other follow-up parameters were observed. In patients treated with a TTR-stabilizer for 24 [20 to 30] months, H/WB ratio increased from 4.46 [3.24 to 5.13] to 4.96 [3.39 to 5.80] (p =.010), and troponin T increased from 19.5 [9.3 to 34.0] ng/L to 20.0 [11.8 to 47.8] ng/L (p =.025). All other parameters did not change during treatment with a TTR-stabilizer. Conclusion: A change in cardiac tracer uptake on bone scintigraphy may be an early marker of treatment-specific response or disease progression in ATTRv amyloidosis patients.

Original languageEnglish
Pages (from-to)681-690
Number of pages10
JournalEuropean journal of nuclear medicine and molecular imaging
Volume51
Issue number3
DOIs
Publication statusPublished - Feb 2024

Keywords

  • ATTR-CM
  • ATTRv amyloidosis
  • Bone scintigraphy
  • Gene silencing
  • Monitoring
  • TTR stabilizers

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