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Objective <p>Tafamidis has shown potential in slowing disease progression in patients with transthyretin amyloid cardiomyopathy (ATTR-CM). This study aimed to evaluate serial changes on [<sup>99m</sup>Tc]Tc-pyrophosphate (PYP) scintigraphy during tafamidis treatment for hereditary ATTR-CM.</p> Methods <p>We retrospectively analyzed a prospectively collected cohort of Ala97Ser (A97S) hereditary ATTR-CM patients treated with tafamidis (61&#xa0;mg/day) and a control group comprising A97S hereditary ATTR-CM patients who had not received disease-modifying medications. The tafamidis group was further divided into two cohorts: cohort A received [<sup>99m</sup>Tc]Tc-PYP SPECT/CT scans at baseline, 1 year, and 2 years; cohort B at baseline, 2 years, and 3 years. Visual score, planar heart to contralateral lung (H/CL) ratio, and volumetric heart to lung (H/L) ratio were measured.</p> Results <p>Nineteen patients were enrolled in the tafamidis group and nine in the control group. After 2 years of follow-up, a significant decrease in volumetric H/L ratio (3.86 ± 0.91 to 3.01 ± 0.19, <i>p</i> &lt; 0.001) was noted in the tafamidis group, while there was no significant change in the control group. When evaluated over time, a significant decrease in volumetric H/L ratio was observed during the first year of tafamidis treatment (3.75 ± 0.37 to 2.82 ± 0.15, <i>p</i> = 0.004), followed by stable [<sup>99m</sup>Tc]Tc-PYP uptake in the subsequent two years (2.82 ± 0.15 to 2.83 ± 0.18, <i>p</i> = 0.934 and 3.20 ± 0.14 to 3.09 ± 0.16, <i>p</i> = 0.404, respectively).</p> Conclusion <p>A significant reduction in [<sup>99m</sup>Tc]Tc-PYP uptake was observed in hereditary ATTR-CM patients after tafamidis treatment, particularly within the first year. While the effect appeared to be sustained, stable [<sup>99m</sup>Tc]Tc-PYP uptake without further significant reductions was observed in the subsequent years.</p>

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Impact of Tafamidis on [99mTc]Tc-pyrophosphate Scintigraphy in Ala97Ser Hereditary Transthyretin amyloid cardiomyopathy: significant initial reduction with stable Long-Term effects

  • 期刊论文

  • 发表日期：

    2025年1月13日

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  • An-Li Yu,
  • Yi-Chieh Chen,
  • Cheng-Hsuan Tsai,
  • Chi-Chao Chao,
  • Mao-Yuan Su,
  • Chia-Tung Shun,
  • Hsueh-Wen Hsueh,
  • Jyh-Ming Jimmy Juang,
  • Ming-Jen Lee,
  • Ping-Huei Tseng,
  • Sung-Tsang Hsieh,
  • Mei-Fang Cheng,
  • Yen-Hung Lin

摘要

Objective

Tafamidis has shown potential in slowing disease progression in patients with transthyretin amyloid cardiomyopathy (ATTR-CM). This study aimed to evaluate serial changes on [99mTc]Tc-pyrophosphate (PYP) scintigraphy during tafamidis treatment for hereditary ATTR-CM.

Methods

We retrospectively analyzed a prospectively collected cohort of Ala97Ser (A97S) hereditary ATTR-CM patients treated with tafamidis (61 mg/day) and a control group comprising A97S hereditary ATTR-CM patients who had not received disease-modifying medications. The tafamidis group was further divided into two cohorts: cohort A received [99mTc]Tc-PYP SPECT/CT scans at baseline, 1 year, and 2 years; cohort B at baseline, 2 years, and 3 years. Visual score, planar heart to contralateral lung (H/CL) ratio, and volumetric heart to lung (H/L) ratio were measured.

Results

Nineteen patients were enrolled in the tafamidis group and nine in the control group. After 2 years of follow-up, a significant decrease in volumetric H/L ratio (3.86 ± 0.91 to 3.01 ± 0.19, p < 0.001) was noted in the tafamidis group, while there was no significant change in the control group. When evaluated over time, a significant decrease in volumetric H/L ratio was observed during the first year of tafamidis treatment (3.75 ± 0.37 to 2.82 ± 0.15, p = 0.004), followed by stable [99mTc]Tc-PYP uptake in the subsequent two years (2.82 ± 0.15 to 2.83 ± 0.18, p = 0.934 and 3.20 ± 0.14 to 3.09 ± 0.16, p = 0.404, respectively).

Conclusion

A significant reduction in [99mTc]Tc-PYP uptake was observed in hereditary ATTR-CM patients after tafamidis treatment, particularly within the first year. While the effect appeared to be sustained, stable [99mTc]Tc-PYP uptake without further significant reductions was observed in the subsequent years.