Ascending aortic remodelling in Fabry disease after long-term enzyme replacement therapy.

Details

Ressource 1Download: smw_147_w14517.pdf (823.94 [Ko])
State: Public
Version: author
License: Not specified
Serval ID
serval:BIB_07EEEE6624BD
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Ascending aortic remodelling in Fabry disease after long-term enzyme replacement therapy.
Journal
Swiss medical weekly
Author(s)
Monney P., Qanadli S.D., Hajdu S., Tran C., Schwitter J., Dormond O., Barbey F.
ISSN
1424-3997 (Electronic)
ISSN-L
0036-7672
Publication state
Published
Issued date
2017
Peer-reviewed
Oui
Volume
147
Pages
w14517
Language
english
Notes
Publication types: Journal Article
Publication Status: epublish
Abstract
Previous cross-sectional studies reported a high prevalence of ascending aorta dilations/aneurysms in male adults with Fabry disease, independently of cardiovascular risk factors.
To characterise the remodelling of the ascending aorta in classic Fabry disease under long-term enzyme replacement therapy.
Diameter of the ascending aorta was measured with magnetic resonance imaging at the sino-tubular junction (STJ), and proximal (pAsAo), and distal ascending aorta (dAsAo) at baseline, and after 5 and 10 years of enzyme replacement therapy in 15 adult Fabry patients (10 males; 5 females).
Over a mean follow-up of 9.5 years, the annual expansion rates measured in 10 males with Fabry disease were 0.41 ± 0.16, 0.36 ± 0.25 and 0.41 ± 0.26 mm/y at the STJ, pAsAo and dAsAo, respectively. Expansion rate at the pAsAo level in male patients was significantly higher than the expected expansion projected from theoretical normal values: 0.36 ± 0.25 vs 0.13 ± 0.05, p = 0.017. In 5 females, the annual expansion rates at the STJ, pAsAo and dAsAo were 0.14 ± 0.11, 0.21 ± 0.18 and 0.26 ± 0.24 mm/y, respectively. There was no significant difference from the projected normal expansion rate at the level of the pAsAo: 0.21 ± 0.18 vs 0.13 ± 0.04, p = 0.39.
Our data suggest that the remodelling of the ascending aorta is more pronounced in male patients with Fabry disease under long-term enzyme replacement therapy compared with the progression observed in a large population study.
Keywords
Adult, Aorta/diagnostic imaging, Aorta/pathology, Aortic Aneurysm/pathology, Dilatation, Disease Progression, Enzyme Replacement Therapy/methods, Fabry Disease/complications, Fabry Disease/genetics, Female, Humans, Longitudinal Studies, Male, Prospective Studies, Risk Factors, Sex Factors
Pubmed
Web of science
Open Access
Yes
Create date
15/11/2017 12:39
Last modification date
25/12/2019 6:18
Usage data