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Performance of 99mTc-PYP scintigraphy in the diagnosis of hereditary transthyretin cardiac amyloidosis

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Abstract

Objective

Most reported research has primarily investigated wild-type transthyretin cardiac amyloidosis (ATTRwt-CA). However, the application of bone scintigraphy for hereditary transthyretin cardiac amyloidosis (ATTRv-CA) has not been systematically investigated. Therefore, in this study, we aimed to evaluate the diagnostic value of 99mTc-PYP scintigraphy in ATTRv-CA.

Methods

Fifty-four patients were enrolled in a highly suspected cardiac amyloidosis cohort. Transthyretin (TTR) gene characteristics were summarized in the ATTRv-CA group. In 99mTc-PYP scintigraphy, the diagnostic efficiency of the visual score (VGS) and heart-to-contralateral chest (H/CL) ratio were evaluated. Furthermore, the interobserver consistency among the diagnosticians was investigated.

Results

Twenty-eight patients were diagnosed with ATTRv-CA with eight genotypes. The Ala97Ser genotype accounts for 46% (n = 13) with a mean age of disease onset, definite diagnosis, and interval of 61.6 ± 1.9, 66.5 ± 1.3, and 4.0 (3.0, 6.2) years, respectively. Their VGS is Grade 3, and their H/CL ratio is higher than that of the non-Ala97Ser group, but no statistical significance exists (mean H/CL: 1.95 ± 0.06 vs. 1.87 ± 0.02, p = 0.844). Additionally, ATTRv-CA patients showed VGS ≥ 2, and mean H/CL ratio of 2.09 ± 0.06. The sensitivity and specificity of VGS were 100% and 65%, respectively. And the interobserver consistency analysis of VGS showed the intraclass correlation coefficient is 0.522. The best cutoff value of H/CL ratio was 1.51 (AUC = 0.996), and the diagnostic consistency of H/CL (bias: 0.018) was high.

Conclusions

Ala97Ser is the most common genotype in ATTRv-CA in our cohort, with characteristics of later onset and rapid progression, but delayed diagnosis and extensive 99mTc-PYP uptake. Overall, ATTRv-CA patients showed moderate-to-extensive myocardial 99mTc-PYP uptake. Additionally, VGS carries subjectivity, low specialty and interobserver consistency. But H/CL exhibit high diagnostic efficacy and interobserver consistency. The H/CL ratio is more useful than VGS.

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Data availability

Data are available on request to the first author.

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Acknowledgements

We express our appreciation to Mrs. Alisa Rogoff from the USA for language editing of the manuscript. The authors declare that the research was conducted without any commercial or financial relationships that could be construed as potential conflicts of interest.

Funding

This work was supported by a significant grant from the Research and Development Program of Hunan Province of China (2019SK2252, 2022SK2035) and the Natural Science Foundation of Hunan Province of China (2020JJ4801).

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Authors and Affiliations

  1. Department of Nuclear Medicine, The Second **angya Hospital, Central South University, Changsha, 410011, Hunan, China

    Honghui Guo, **n **ang, Zhihui Fang, Qianchun Ye, Yao Zou, Yunhua Wang & **aowei Ma

  2. Department of Cardiovascular Medicine, The Second **angya Hospital of Central South University, Changsha, 410011, Hunan, China

    Sha Wu, Shuai Wang & Daoquan Peng

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  1. Honghui Guo
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Guo, H., Wu, S., **ang, X. et al. Performance of 99mTc-PYP scintigraphy in the diagnosis of hereditary transthyretin cardiac amyloidosis. Ann Nucl Med 38, 288–295 (2024). https://doi.org/10.1007/s12149-023-01898-x

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