Artery Research

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Volume 3, Issue 4, December 2009, Pages 153 - 154

3.3 EFFECT OF CELIPROLOL ON PREVENTION OF CARDIOVASCULAR EVENTS IN VASCULAR EHLERS-DANLOS SYNDROME

Authors
K.T. Ong1, J. Perdu2, H. Plauchu3, J. De Backer4, A. De Paepe4, J. Emmerich2, X. Jeunemaitre5, D. Germain6, P. Collignon7, G. Georgesco8, E. Bozec1, J.S. Hulot9, S. Laurent1, P. Boutouyrie1
1Department of Pharmacology and INSERM U970, Georges Pompidou European Hospital, Paris, France
2Department of Vascular Medicine, Georges Pompidou European Hospital, Paris, France
3Department of Genetics, Hôtel Dieu Hospital, Lyon, France
4Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium
5Department of Genetics, Georges Pompidou European Hospital, Paris, France
6Department of Genetics, Raymond Poincaré Hospital, Garches, France
7Department of Genetics, the Timone Hospital, Marseille, France
8Department of Dermatology, Trousseau Hospital, Tours, France
9Department of Pharmacology, Pitié Salpêtrière Hospital, Paris, France
Available Online 3 December 2009.
DOI
10.1016/j.artres.2009.10.154How to use a DOI?
Abstract

Background: Vascular Ehlers-Danlos syndrome (vEDS) is a rare severe genetic disease which results from mutations in the gene encoding type III procollagen (COL3A1), characterized by vascular and/or hollow organic ruptures. No treatment is yet validated. We tested the ability of celiprolol, a beta1-adrenoceptor antagonist with a beta2-adrenoceptor agonist action, for preventing the complications of vEDS in a prospective, randomized, open, blinded endpoints trial.

Methods: Fifty three previously untreated vEDS patients were randomized to a 5-year treatment with either celiprolol (n=25) or no treatment (n=28). The two groups were matched for demographic, medical historic and clinical characteristics. Celiprolol was up-titrated from 100 to 400mg by steps of 100mg every 6 months. The primary end-point was an arterial event (rupture or dissection, fatal or not) occurring during follow-up. Secondary endpoints were intestinal or uterine rupture or major clinical events, related to vEDS, judged by the event committee.

Results: Mean duration of follow-up was 47 (± 15) months. The study was ended prematurely by the safety monitoring board since significant differences were reached between two groups. The primary endpoint was reached by 5 patients (20%) in the celiprolol group and by 14 patients (50%) in the control group (hazard ratio, 0.36; 95% CI, 0.15 to 0.88; P=0.04). Primary plus secondary endpoints occurred in 6 patients (24%) in the celiprolol group and in 17 patient (61%) in the control group (hazard ratio, 0.31; 95% CI, 0.14 to 0.71; P=0.0097).

Conclusions: Celiprolol effectively reduced both vascular complications and organic ruptures in vEDS patients.

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Journal
Artery Research
Volume-Issue
3 - 4
Pages
153 - 154
Publication Date
2009/12/03
ISSN (Online)
1876-4401
ISSN (Print)
1872-9312
DOI
10.1016/j.artres.2009.10.154How to use a DOI?
Open Access
This is an open access article distributed under the CC BY-NC license.

Cite this article

risenwbib
TY  - JOUR
AU  - K.T. Ong
AU  - J. Perdu
AU  - H. Plauchu
AU  - J. De Backer
AU  - A. De Paepe
AU  - J. Emmerich
AU  - X. Jeunemaitre
AU  - D. Germain
AU  - P. Collignon
AU  - G. Georgesco
AU  - E. Bozec
AU  - J.S. Hulot
AU  - S. Laurent
AU  - P. Boutouyrie
PY  - 2009
DA  - 2009/12/03
TI  - 3.3 EFFECT OF CELIPROLOL ON PREVENTION OF CARDIOVASCULAR EVENTS IN VASCULAR EHLERS-DANLOS SYNDROME
JO  - Artery Research
SP  - 153
EP  - 154
VL  - 3
IS  - 4
SN  - 1876-4401
UR  - https://doi.org/10.1016/j.artres.2009.10.154
DO  - 10.1016/j.artres.2009.10.154
ID  - Ong2009
ER  -