PT  - JOURNAL ARTICLE
AU  - Van Der Gucht, Axel
AU  - Galat, Arnault
AU  - Itti, Emmanuel
AU  - Guellich, Aziz
AU  - Rappeneau, Stephane
AU  - Dubois-Randé, Jean-Luc
AU  - Plante-Bordeneux, Violaine
AU  - Meignan, Michel
AU  - Rosso, Jean
AU  - Damy, Thibaud
TI  - <sup>99m</sup>Tc HMDP myocardial scintigraphy is predictive of major adverse cardiac event (MACE) in patients with transthyretin-type (TTR) amyloidosis
DP  - 2015 May 01
TA  - Journal of Nuclear Medicine
PG  - 27--27
VI  - 56
IP  - supplement 3
4099  - http://jnm.snmjournals.org/content/56/supplement_3/27.short
4100  - http://jnm.snmjournals.org/content/56/supplement_3/27.full
SO  - J Nucl Med2015 May 01; 56
AB  - 27 Objectives Amyloidosis is characterized by extracellular deposits of insoluble proteins with 3 main types: monoclonal light chain (AL), wild-type transthyretin (wt-TTR), and mutated transthyretin (m-TTR). We evaluated the prognostic value of 99mTc-HMDP myocardial scintigraphy in patients with TTR amyloidosis.Methods Out of 121 consecutive patients, 55 patients were diagnosed with TTR amyloidosis. All underwent 99mTc-HMDP scintigraphy using a dual-headed gamma camera. Acquisitions consisted of a whole-body scan and a cardiac tomography, acquired both at 10 min (early soft-tissue phase) and 3 h (delayed bone phase) following the injection (650 MBq i.v.). Myocardial uptake of 99mTc-HMDP was assessed visually (score 0-3) and semi-quantitatively (heart-to-skull retention [HR/SR]).Results Among 55 TTR patients, 47 had cardiac amyloidosis (wt-TTR-CA in 21 and m-TTR-CA in 26 patients). Delayed myocardial 99mTc- HMDP uptake occurred in all wt-TTR-CA, in all but 2 m-TTR-CA. All patients with TTR-CA had a positive HMDP myocardial scintigraphy. Except 1 wt-TTR-CA and 5 m-TTR-CA who had a score of 1, all others had a score ≥2. To note, scintigraphy was negative in the 8 patients without cardiac amyloidosis at early and delayed phase. Among 55 TTR patients, HR/SR ratio was correlated with CA severity (LVEF and NT-proBNP). Median follow-up was 111 d (50-343). Patients with HR/SR<1.94 had better 6-mo MACE-free survival (95%) than those with HR/SR>1.94 (60%, P=0.025). Interestingly, combining NYHA class with HR/SR ratio allowed to identify prognostic groups of patients ranging from 100% MACE-free survival to 44%.Conclusions 99mTc-HMDP myocardial scintigraphy is predictive of MACE-free survival in TTR amyloidosis, particularly in combination with NYHA classification.