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Purpose <p>The relevance of repetitive [<sup>99m</sup>Tc]Tc-DPD scintigraphy in wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM) remains unclear. We investigated the impact of tafamidis on cardiac [<sup>99m</sup>Tc]Tc-DPD uptake, clinical, and laboratory markers at 6 and 12&#xa0;months, and correlated 12&#xa0;months [<sup>99m</sup>Tc]Tc-DPD uptake regression with survival.</p> Methods <p>This single-center study enrolled 39 ATTRwt-CM patients. Upon treatment initiation with tafamidis, patients underwent follow-up [<sup>99m</sup>Tc]Tc-DPD scintigraphy, and clinical and laboratory evaluations at 6&#xa0;months (<i>n</i> = 6) and 12&#xa0;months (<i>n</i> = 13), or both (<i>n</i> = 20).</p> Results <p>Tafamidis resulted in a significant decline in Perugini score (6&#xa0;months <i>p</i> = 0.008, 12&#xa0;months <i>p</i> &lt; 0.001), and (semi-)quantitative [<sup>99m</sup>Tc]Tc-DPD uptake (total cardiac uptake: baseline 816 [522–933]&#xa0;cps, vs. 6&#xa0;months 634 [502–734]&#xa0;cps, <i>p</i> = 0.003, vs. 12&#xa0;months 523 [108–754]&#xa0;cps, <i>p</i> = 0.001). Clinical and laboratory improvements were observed (NYHA: 6&#xa0;months <i>p</i> = 0.007, 12&#xa0;months <i>p</i> = 0.033; NT-proBNP: baseline 2586 [1271–5561]&#xa0;ng/L, vs. 6&#xa0;months 2526 [1109–4786]&#xa0;ng/L, <i>p</i> = 0.016, vs. 12&#xa0;months 2340 [1411–4749]&#xa0;ng/L, <i>p</i> = 0.012). In Kaplan–Meier analysis, a decrease in right ventricular [<sup>99m</sup>Tc]Tc-DPD tracer uptake equal to or greater than the median value at 12&#xa0;months (-30%) was associated with improved survival (log-rank <i>p</i> = 0.021).</p> Conclusions <p>Tafamidis in ATTRwt-CM resulted in significant reductions of cardiac [<sup>99m</sup>Tc]Tc-DPD uptake, NYHA class, and cardiac biomarkers at 6 and 12&#xa0;months. Regression of right ventricular [<sup>99m</sup>Tc]Tc-DPD uptake at 12&#xa0;months was associated with improved survival.</p>

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Effects of tafamidis on serial [99mTc]Tc-DPD scintigraphy in transthyretin amyloid cardiomyopathy

  • 期刊论文

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  • 发表日期：

    2025年2月6日

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  • Maria Ungericht,
  • Thomas Schuetz,
  • Moritz Messner,
  • Christian Puelacher,
  • Simon Staggl,
  • Marc-Michael Zaruba,
  • Alexander Stephan Kroiss,
  • Axel Bauer,
  • Gerhard Poelzl

摘要

Purpose

The relevance of repetitive [99mTc]Tc-DPD scintigraphy in wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM) remains unclear. We investigated the impact of tafamidis on cardiac [99mTc]Tc-DPD uptake, clinical, and laboratory markers at 6 and 12 months, and correlated 12 months [99mTc]Tc-DPD uptake regression with survival.

Methods

This single-center study enrolled 39 ATTRwt-CM patients. Upon treatment initiation with tafamidis, patients underwent follow-up [99mTc]Tc-DPD scintigraphy, and clinical and laboratory evaluations at 6 months (n = 6) and 12 months (n = 13), or both (n = 20).

Results

Tafamidis resulted in a significant decline in Perugini score (6 months p = 0.008, 12 months p < 0.001), and (semi-)quantitative [99mTc]Tc-DPD uptake (total cardiac uptake: baseline 816 [522–933] cps, vs. 6 months 634 [502–734] cps, p = 0.003, vs. 12 months 523 [108–754] cps, p = 0.001). Clinical and laboratory improvements were observed (NYHA: 6 months p = 0.007, 12 months p = 0.033; NT-proBNP: baseline 2586 [1271–5561] ng/L, vs. 6 months 2526 [1109–4786] ng/L, p = 0.016, vs. 12 months 2340 [1411–4749] ng/L, p = 0.012). In Kaplan–Meier analysis, a decrease in right ventricular [99mTc]Tc-DPD tracer uptake equal to or greater than the median value at 12 months (-30%) was associated with improved survival (log-rank p = 0.021).

Conclusions

Tafamidis in ATTRwt-CM resulted in significant reductions of cardiac [99mTc]Tc-DPD uptake, NYHA class, and cardiac biomarkers at 6 and 12 months. Regression of right ventricular [99mTc]Tc-DPD uptake at 12 months was associated with improved survival.