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Reduction in 99mTc-DPD myocardial uptake with therapy of ATTR cardiomyopathy.

Authors :
Rettl, René
Calabretta, Raffaella
Duca, Franz
Binder, Christina
Kronberger, Christina
Willixhofer, Robin
Poledniczek, Michael
Donà, Carolina
Nitsche, Christian
Beitzke, Dietrich
Loewe, Christian
Auer-Grumbach, Michaela
Bonderman, Diana
Kastl, Stefan
Hengstenberg, Christian
Badr Eslam, Roza
Kastner, Johannes
Bergler-Klein, Jutta
Hacker, Marcus
Kammerlander, Andreas
Source :
Amyloid; Mar2024, Vol. 31 Issue 1, p42-51, 10p
Publication Year :
2024

Abstract

Aims: Novel ribonucleic acid interference (RNAi) therapeutics such as patisiran and inotersen have been shown to benefit neurologic disease course and quality of life in patients with hereditary transthyretin amyloidosis (ATTRv). We aimed to determine the impact of RNAi therapeutics on myocardial amyloid load using quantitative single photon emission computed tomography/computed tomography (SPECT/CT) imaging in patients with ATTRv-related cardiomyopathy (ATTRv-CM). We furthermore compared them with wild-type ATTR-CM (ATTRwt-CM) patients treated with tafamidis. Methods and results: ATTRv-CM patients underwent [<superscript>99m</superscript>Tc]-radiolabeled diphosphono-1,2-propanodicarboxylic acid (<superscript>99m</superscript>Tc-DPD) scintigraphy and quantitative SPECT/CT imaging before and after 12 months (IQR: 11.0–12.0) of treatment with RNAi therapeutics (patisiran: n = 5, inotersen: n = 4). RNAi treatment significantly reduced quantitative myocardial uptake as measured by standardised uptake value (SUV) retention index (baseline: 5.09 g/mL vs. follow-up: 3.19 g/mL, p =.028) in ATTRv-CM patients without significant improvement in cardiac function. Tafamidis treatment resulted in a significant reduction in SUV retention index (4.96 g/mL vs. 3.27 g/mL, p <.001) in ATTRwt-CM patients (historical control cohort: n = 40) at follow-up [9.0 months (IQR: 7.0–10.0)] without beneficial impact on cardiac function. Conclusions: RNAi therapeutics significantly reduce quantitative myocardial uptake in ATTRv-CM patients, comparable to tafamidis treatment in ATTRwt-CM patients, without impact on cardiac function. Serial <superscript>99m</superscript>Tc-DPD SPECT/CT imaging may be a valuable tool to quantify and monitor response to disease-specific therapies in both ATTRv-CM and ATTRwt-CM. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
13506129
Volume :
31
Issue :
1
Database :
Complementary Index
Journal :
Amyloid
Publication Type :
Academic Journal
Accession number :
175643463
Full Text :
https://doi.org/10.1080/13506129.2023.2247136