Volume 10 Supplement 1

First European Congress on Hereditary ATTR amyloidosis

Open Access

Quantitative comparison between amyloid deposition detected by 99mTc-diphosphonate imaging and myocardial deformation evaluated by strain echocardiography in transthyretin related cardiac amyloidosis

  • Gianluca Di Bella1,
  • Fabio Minutoli2,
  • Anna Mazzeo3,
  • Claudia Stancanelli3,
  • Luca Gentile3,
  • Sergio Baldari2,
  • Scipione Carerj1 and
  • Giuseppe Vita3
Orphanet Journal of Rare Diseases201510(Suppl 1):P47

https://doi.org/10.1186/1750-1172-10-S1-P47

Published: 2 November 2015

Aims

The aim of our study is to assess the effect of amyloid deposition on myocardial function.

Methods and results

28 patients with transthyretin mutation and a group of 14 controls underwent echocardiography to quantify left ventricular (LV) dimensions and function, and global (G) longitudinal (L), radial (R) and circumferential (C) strain (S). 99mTc-3, 3-diphosphono-1, 2-propanodicarboxylic-acid-scintigraphy (99mTc-DPD) was used to quantify cardiac amyloidosis (CA). 99mTc-DPD revealed accumulation in 14 of 28 patients (CA-group) and no accumulation (no CA-group) in 14 patients. Cardiac accumulation was mild-moderate in 5 (Mild-Moderate CA-group) and severe in 9 patients (Severe CA-group). Severe CA-group showed higher values of LV septal thickness (LVST), posterior wall thickness and E/E’ ratio than the no CA-group and the control group (adj. p<0.05). Ejection fraction was similar among groups (p=0.65). GLS was lower (p=<0.001) in severe CA-group (-12.2 ± 4.5) respect to no CA-group (-19.3 ± 3.0) and to the control group (-20.9 ± 2.5). On the contrary, GCS and GRS were lower (p=<0.05) in mild-moderate CA-group (-10.8 ± 4.1 and 9.5 ± 5.7, respectively) respect to the severe CA-group (-18.9 ± 5.1 and 23.9 ± 6.3 respectively), no CA-group (-19.2 ± 4.1 and 28.4 ± 10.2 respectively) and the control group (-23.9 ± 4.4 and 29.9 ± 8.7 respectively). A correlation was found between the scintigraphic heart retention (HR) index and LVST (ρ=0.72; p<0.001) and E/E’ (ρ=0.46; p=0.03). An inverse tendency was observed between HR and GLS (ρ=−0.40; p=0.06).

Conclusions

99mTc-DPD HR is well correlated with LVST, diastolic and longitudinal systolic dysfunction.

Authors’ Affiliations

(1)
Clinical and experimental medicine department, University of Messina
(2)
Department of Biomedical Sciences and of Morphologic and Functional Images, University of Messina
(3)
Department of Neurosciences, University of Messina

Copyright

© Di Bella et al. 2015

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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