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