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Purpose <p>To determine whether <i>TTRv</i> carriers with Perugini grade 1 cardiac radiotracer uptake on [<sup>99m</sup>Tc]Tc- hydroxydiphosphonate bone scintigraphy have or develop ATTR-CM.</p> Methods <p>This retrospective observational study was conducted at the Groningen Amyloidosis Centre of Expertise between April 2012 and June 2023. <i>TTRv</i> carriers with Perugini grade 1 uptake on bone scintigraphy were followed until to June 2024. Data on symptoms, biomarkers, imaging, and biopsies were collected. A descriptive analysis was performed to evaluate whether carriers met the diagnostic criteria for ATTR-CM or ‘probable ATTR-CM’ at baseline and follow-up.</p> Results <p>Out of 178 <i>TTRv</i> carriers in screening, 12 carriers had Perugini grade 1 cardiac radiotracer uptake on bone scintigraphy. At baseline, 2 carriers met the diagnostic criteria for ATTR-CM and 3 carriers met the criteria for probable ATTR-CM. Of the 7 carriers without (probable) ATTR-CM at baseline, 3 carriers were diagnosed with ATTR-CM during follow-up and 1 carrier developed probable ATTR-CM during follow-up. Three carriers showed signs of cardiomyopathy during follow-up, but did not meet the criteria for (probable) ATTR-CM. One of these cases may have been false-positive due to hydroxychloroquine use.</p> Conclusion <p>Our findings suggest that Perugini grade 1 cardiac radiotracer uptake is an early marker of ATTR-CM in <i>TTRv</i> carriers, potentially enabling earlier diagnosis and intervention.</p>

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Detecting early cardiomyopathy in transthyretin variant carriers: reappraising the diagnostic value of Perugini grade 1 radiotracer uptake on bone scintigraphy

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年5月23日

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  • H. S. A. Tingen,
  • M. Berends,
  • A. Tubben,
  • P. van der Meer,
  • R. H. J. A. Slart,
  • J. Bijzet,
  • P. A. van der Zwaag,
  • C. Kimmich,
  • C. Knackstedt,
  • F. L. H. Muntinghe,
  • E. J. Houwerzijl,
  • B. P. C. Hazenberg,
  • H. L. A. Nienhuis

摘要

Purpose

To determine whether TTRv carriers with Perugini grade 1 cardiac radiotracer uptake on [99mTc]Tc- hydroxydiphosphonate bone scintigraphy have or develop ATTR-CM.

Methods

This retrospective observational study was conducted at the Groningen Amyloidosis Centre of Expertise between April 2012 and June 2023. TTRv carriers with Perugini grade 1 uptake on bone scintigraphy were followed until to June 2024. Data on symptoms, biomarkers, imaging, and biopsies were collected. A descriptive analysis was performed to evaluate whether carriers met the diagnostic criteria for ATTR-CM or ‘probable ATTR-CM’ at baseline and follow-up.

Results

Out of 178 TTRv carriers in screening, 12 carriers had Perugini grade 1 cardiac radiotracer uptake on bone scintigraphy. At baseline, 2 carriers met the diagnostic criteria for ATTR-CM and 3 carriers met the criteria for probable ATTR-CM. Of the 7 carriers without (probable) ATTR-CM at baseline, 3 carriers were diagnosed with ATTR-CM during follow-up and 1 carrier developed probable ATTR-CM during follow-up. Three carriers showed signs of cardiomyopathy during follow-up, but did not meet the criteria for (probable) ATTR-CM. One of these cases may have been false-positive due to hydroxychloroquine use.

Conclusion

Our findings suggest that Perugini grade 1 cardiac radiotracer uptake is an early marker of ATTR-CM in TTRv carriers, potentially enabling earlier diagnosis and intervention.