Celiprolol in Patients With Ehlers-Danlos Syndrome, Vascular Type

Study Overview
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Brief Summary
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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
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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.

Official Title
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Prevention of Vascular Complications by BetaBlocker Treatment in Vascular Ehlers-Danlos Syndrome

Conditions
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EHLERS-DANLOS SYNDROME, TYPE IV, AUTOSOMAL DOMINANT, CHROMOSOME 2q31.2 DELETION SYNDROME

Intervention / Treatment
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* Drug: celiprolol
* Drug: Control


Participation Criteria
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Eligibility Criteria
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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.

Ages Eligible for Study
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Minimum Age: 15 Years
Maximum Age: 65 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Allocation: Randomized
Intervention Model: Parallel Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Treatment<br>Celiprolol | Drug: celiprolol<br>* celiprolol<br>* Other names: every 6 months by increment of 100 mg, based on tolerance;Drug: Control<br>* Untreated controls excluding betablockers<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| reduction in the rate of major cardiovascular events in the treated group during a 5 years follow-up | reduction in the rate of major cardiovascular events in the treated group during a 5 years follow-up | during de study | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Tolerance, effect of drug on arterial parameters : diameter, IMT, stiffness. | Tolerance, effect of drug on arterial parameters : diameter, IMT, stiffness. | during the study | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Arteries, ultrasonography, Ehlers Danlos syndrome, Cardiovascular disease, beta adrenergic antagonists