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Usefulness of 99mTc-HMDP scintigraphy for the etiologic diagnosis and prognosis of cardiac amyloidosis

Background

Amyloidosis is characterized by extracellular deposits of insoluble proteins that cause tissue damage. The three main types are monoclonal light chain (AL), wild-type transthyretin (wt-TTR), and mutated transthyretin (m-TTR) amyloidosis. Cardiac amyloidosis (CA) raises diagnostic challenges.

Objective

To assess the diagnostic accuracy of 99mTc-HMDP-scintigraphy for typing CA, differentiating CA from non-amyloid left ventricle hypertrophy (LVH), and predicting outcomes.

Methods

121 patients with suspected CA underwent 99mTc-HMDP-scintigraphy in addition to standard investigations.

Results

CA was diagnosed in all AL (n=14) and wt-TTR (n=21). Among m-TTR (n=34), 26 had CA, 4 neuropathy without CA and 4 were asymptomatic carriers. Of the 52 patients with non-amyloid heart disease, 37 had LVH and served as controls. 99mTc-HMDP cardiac uptake occurred in all wt-TTR, in m-TTR with CA except two, and in one AL. A visual score ≥2 was 100% specific for diagnosing TTR-CA. Among TTR-CA, heart-to-skull retention (HR/SR) correlated with CA severity (LVEF and NT-proBNP). Median follow-up was 111 days (50;343). In a multivariate Cox model including clinical, echocardiographic, and scintigraphic variables, NYHA III-IV and HR/SR>1.94 predicted acute heart failure and/or death.

Conclusions

99mTc-HMDP-scintigraphy allows differentiating transthyretin from AL-CA and CA from other LVHs and also provides prognostic information.

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Correspondence to Arnault Galat.

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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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Galat, A., Rosso, J., Guellich, A. et al. Usefulness of 99mTc-HMDP scintigraphy for the etiologic diagnosis and prognosis of cardiac amyloidosis. Orphanet J Rare Dis 10, P49 (2015). https://doi.org/10.1186/1750-1172-10-S1-P49

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Keywords

  • Neuropathy
  • Amyloidosis
  • Acute Heart Failure
  • Asymptomatic Carrier
  • Cardiac Amyloidosis