Celiprolol in Patients With Ehlers-Danlos Syndrome, Vascular Type

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

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

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.

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.