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Published in: European Journal of Nuclear Medicine and Molecular Imaging 10/2020

01-09-2020 | Cardiac Amyloidosis | Original Article

Extracardiac soft tissue uptake, evidenced on early 99mTc-HMDP SPECT/CT, helps typing cardiac amyloidosis and demonstrates high prognostic value

Authors: Nathan Malka, Mukedaisi Abulizi, Mounira Kharoubi, Silvia Oghina, Arnault Galat, Fabien Le Bras, Anissa Moktefi, Soulef Guendouz, Valérie Molinier-Frenkel, Pascale Fanen, Benoît Funalot, Jean-Pascal Lefaucheur, Paul Blanc-Durand, Jean-François Deux, Vincent Audard, Diane Bodez, Emmanuel Itti, Thibaud Damy

Published in: European Journal of Nuclear Medicine and Molecular Imaging | Issue 10/2020

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Abstract

Purpose

Increased cardiac uptake (CU) on early-phase 99mTc-HMDP scintigraphy has demonstrated diagnostic and prognostic values in amyloid transthyretin (ATTR) cardiac amyloidosis (CA). Extracardiac uptake (ECU) has been poorly studied. We assessed the clinical value of ECU, in combination with CU, on 99mTc-HMDP scintigraphy using a novel Methodological Amyloidosis Diagnostic Index (MADI).

Methods

We reviewed all patients referred for suspicion of CA, who underwent 99mTc-HMDP scintigraphy over an 8-year period. ECU, CU, and MADI were determined: MADI0 = neither ECU or CU, MADI1 = ECU alone, MADI2 = CU alone, and MADI3 = ECU + CU.

Results

Of 308 eligible patients, 247 had CA, including 75 ATTRv, 107 ATTRwt, and 65 light-chain (AL), while 61 had another cardiopathy (controls). ECU was observed in 29% of CA and 3% of controls. Most frequent sites of ECU were pleuropulmonary (16% of CA, 3% of controls) followed by the digestive tract and subcutaneous tissues. The liver and spleen ECU was only observed in AL-CA (n = 8). CU was only observed in CA patients (n = 187), of whom 182 had ATTR-CA vs. 5 AL-CA, P < 0.001. MADI0 was only observed in controls (97%) and in AL-CA (60%). MADI1 was mainly observed in AL-CA (positive predictive value, PPV = 91%) while MADI2/3 were more frequent in ATTR-CA (PPV = 97%), P < 0.0001. MADI > 0 vs. MADI0 in AL and MADI3 vs. MADI2 in ATTR were associated with a worse prognosis (P = 0.03 and P = 0.002, respectively).

Conclusions

ECU combined with CU demonstrates high diagnostic and prognostic values in CA patients. MADI seems an easy and reliable score in clinical practice.
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Literature
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go back to reference Damy T, Kristen AV, Suhr OB, Maurer MS, Plante-Bordeneuve V, Yu CR, et al. Transthyretin cardiac amyloidosis in continental Western Europe: an insight through the Transthyretin Amyloidosis Outcomes Survey (THAOS). Eur Heart J. 2019;5425268. https://doi.org/10.1093/eurheartj/ehz173. Damy T, Kristen AV, Suhr OB, Maurer MS, Plante-Bordeneuve V, Yu CR, et al. Transthyretin cardiac amyloidosis in continental Western Europe: an insight through the Transthyretin Amyloidosis Outcomes Survey (THAOS). Eur Heart J. 2019;5425268. https://​doi.​org/​10.​1093/​eurheartj/​ehz173.
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go back to reference Kristen AV, Maurer MS, Rapezzi C, Mundayat R, Suhr OB, Damy T. Impact of genotype and phenotype on cardiac biomarkers in patients with transthyretin amyloidosis - report from the Transthyretin Amyloidosis Outcome Survey (THAOS). PLoS One. 2017;12:e0173086.CrossRef Kristen AV, Maurer MS, Rapezzi C, Mundayat R, Suhr OB, Damy T. Impact of genotype and phenotype on cardiac biomarkers in patients with transthyretin amyloidosis - report from the Transthyretin Amyloidosis Outcome Survey (THAOS). PLoS One. 2017;12:e0173086.CrossRef
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Metadata
Title
Extracardiac soft tissue uptake, evidenced on early 99mTc-HMDP SPECT/CT, helps typing cardiac amyloidosis and demonstrates high prognostic value
Authors
Nathan Malka
Mukedaisi Abulizi
Mounira Kharoubi
Silvia Oghina
Arnault Galat
Fabien Le Bras
Anissa Moktefi
Soulef Guendouz
Valérie Molinier-Frenkel
Pascale Fanen
Benoît Funalot
Jean-Pascal Lefaucheur
Paul Blanc-Durand
Jean-François Deux
Vincent Audard
Diane Bodez
Emmanuel Itti
Thibaud Damy
Publication date
01-09-2020
Publisher
Springer Berlin Heidelberg
Published in
European Journal of Nuclear Medicine and Molecular Imaging / Issue 10/2020
Print ISSN: 1619-7070
Electronic ISSN: 1619-7089
DOI
https://doi.org/10.1007/s00259-020-04753-7

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