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

Authors :
Aziz Guellich
Axel Van Der Gucht
Jean-Luc Dubois-Randé
Jean Rosso
Emmanuel Itti
Thibaud Damy
Arnault Galat
Violaine Planté-Bordeneuve
Source :
Orphanet Journal of Rare Diseases
Publication Year :
2015
Publisher :
BioMed Central, 2015.

Abstract

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.

Details

Language :
English
ISSN :
17501172
Volume :
10
Issue :
Suppl 1
Database :
OpenAIRE
Journal :
Orphanet Journal of Rare Diseases
Accession number :
edsair.doi.dedup.....521df25f22f45b9d753bb53d888d5235