Volume 10 Supplement 1

First European Congress on Hereditary ATTR amyloidosis

Open Access

Comparison and identification of early clinical, biological and echocardiographic prognostic markers in cardiac amyloidosis

  • Thibaud Damy1,
  • Arnaud Jaccard2,
  • Aziz Guellich1,
  • David Lavergne2,
  • François Deux Jean1,
  • Jehan Dupuis1,
  • Valérie Frenkel1 and
  • Dania Mohty2
Orphanet Journal of Rare Diseases201510(Suppl 1):P60

https://doi.org/10.1186/1750-1172-10-S1-P60

Published: 2 November 2015

Background

The early prognosis of amyloidosis is known to depend heavily on cardiac function and may be improved by identifying patients at highest risk for adverse cardiac events. We looked for early predictors of mortality in patients with cardiac AL amyloidosis, hereditary transthyretin amyloidosis (m-TTR), or senile transthyretin amyloidosis (WT-TTR).

Method

Prospective observational study of 198 patients seen at two French university centers.

Results

NYHA class was III-IV in 31% of patients. Median (25th-75th percentile) values were 69 (60-76) years for age, 3027 (673-7155) pg•mL-1 for NT-proBNP, and 60% (48-66) for left ventricular ejection fraction. Interventricular septal thickness was greater in the m-TTR and WT-TTR groups than in the AL group (P<0.0001). NT-proBNP correlated with IVST (R=0.34; P=0.0001). The 6-month mortality rate was 24% (42 patients). The AL group had higher values for both NT-proBNP (P=0.0001) and 6-month mortality (P=0.0001). By multivariate analysis, independent predictors of 6-month mortality were higher NT-proBNP (Q4), NYHA class (III-IV), lower cardiac output (<4 L.min-1), and pericardial effusion.

Conclusions

NYHA, NT-proBNP, cardiac output, and pericardial effusion were independent predictors of mortality in cardiac disease due to any of the three amyloidosis types. NT-proBNP values were highest in AL amyloidosis.

Authors’ Affiliations

(1)
Amyloidosis Mondor Creteil, CHU H Mondor
(2)
AL Amyloidosis Referral Center, CHU Limoges

Copyright

© Damy 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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