Clinical trial NCT00190411: Celiprolol in Patients With Ehlers-Danlos Syndrome, Vascular Type
Official title: Prevention of Vascular Complications by BetaBlocker Treatment in Vascular Ehlers-Danlos Syndrome
Study type: INTERVENTIONAL
Overall status: COMPLETED
Phase: PHASE4
Conditions: EHLERS-DANLOS SYNDROME, TYPE IV, AUTOSOMAL DOMINANT
CHROMOSOME 2q31.2 DELETION SYNDROME
Keywords: Arteries
ultrasonography
Ehlers Danlos syndrome
Cardiovascular disease
beta adrenergic antagonists
Lead sponsor: Assistance Publique - Hôpitaux de Paris
Sponsor class: OTHER
Collaborators: name: Aventis Pharmaceuticals
First posted: 2005-09-19
Enrollment: 54
Sex: ALL
Minimum age (years): 15
Maximum age (years): 65
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Allocation: RANDOMIZED
Intervention model: PARALLEL
Primary purpose: TREATMENT

Brief summary: Ehlers-Danlos syndrome vascular type (EDS-IV) is caused by a genetic defect of collagen type III. Patient die (median 40 yrs) of vascular complications. There is no treatment. We showed that arteries are thin and overloaded in this patients. We test the protective effect of celiprolol on cardiovascular events in a 5 years, randomized, PROBE design

Detailed description: 100 patients with verified EDS-IV syndrome are included. Patients are randomized to either celiprolol (50 to 400 mg BID)or no treatment. Patients who are not randomized enter a longitudinal survey of events. The hypothesis is a 50% reduction in the occurrence of cardiovascular events under treatment, assessed by a blinded, independent events committee.

Eligibility criteria: Inclusion Criteria:

* Proven disease,
* No betablocker at inclusion if previous CV event

Exclusion criteria:

Criteria of not inclusion for the RIGHTEOUS group:

* Patient having already presented an arterial break or a dissection and treated(handled) by bétâ-blocking(surrounding).
* Against indication in the use of CELIPROLOL:
* Unchecked cardiac insufficiency by the treatment
* cardiogenic shock
* BAV of 2nd and 3rd not sailed degrees
* angor of Prinzmetal
* disease of the sine
* bradycardia
* pheochromocytoma untreated
* low blood pressure
* sentimentality in the CELIPROLOL
* Antecedent of anaphylactic reaction
* myasthenia
* treatment by FLOCTAFENINE ( Idarac), Sultopride ( interactions ) In these two cases, the patient can be included in the group followed by troop.

Criteria of not inclusion for both groups:

* Refusal to participate in the study.
* Impossibility to move.
* Pregnancy
* Woman in age to procreate without means of effective contraception.

Primary outcomes:
measure: reduction in the rate of major cardiovascular events in the treated group during a 5 years follow-up
timeFrame: during de study
Secondary outcomes:
measure: Tolerance, effect of drug on arterial parameters : diameter, IMT, stiffness.
timeFrame: during the study
Other outcomes:
Locations:
Study officials:
affiliation: Assistance Publique - Hôpitaux de Paris
name: Pierre BOUTOUYRIE, MD,PhD
role: PRINCIPAL_INVESTIGATOR
References:
citation: Ong KT, Perdu J, De Backer J, Bozec E, Collignon P, Emmerich J, Fauret AL, Fiessinger JN, Germain DP, Georgesco G, Hulot JS, De Paepe A, Plauchu H, Jeunemaitre X, Laurent S, Boutouyrie P. Effect of celiprolol on prevention of cardiovascular events in vascular Ehlers-Danlos syndrome: a prospective randomised, open, blinded-endpoints trial. Lancet. 2010 Oct 30;376(9751):1476-84. doi: 10.1016/S0140-6736(10)60960-9. Epub 2010 Sep 7.