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May 28 2019

Research News: New scientific publication on Long-Term Observational study of vEDS


A new publication, co-authored by Dr. Michael Frank (Medium-Sized Arteries Working Group (MSA-WG) Co-Chair), Prof. Xavier Jeunemaitre and Dr. Salma Adham (members of the MSA WG) and entitled Vascular Ehlers-Danlos syndrome: Long-Term Observational study, has recently been published in the Journal of the American College of Cardiology.

Vascular Ehlers-Danlos syndrome (vEDS) is one of the most severe forms of Ehlers-Danlos syndrome (EDS) due to its association with life-threatening complications such as spontaneous arterial dissections, aneurysms, and/or ruptures, gastrointestinal perforations and uterine rupture during pregnancy.

This article reports the findings from a long-term follow-up of a large, COL3A1 pathogenic variant–confirmed vEDS patient cohort (recorded in a database at Hôpital Européen Georges Pompidou, AP-HP in Paris, France – one of VASCERN’s 31 HCPs) that was offered treatment with celiprolol, a type of β-blocker medication that has previously shown promising results (i.e. significant reduction of cardiovascular morbidity and mortality in treated patients) in a clinical trial that took place in 2010.

Main findings:

At the end of the study period, almost all patients (90.3%) were treated with celiprolol alone or in combination. More than two-thirds of patients remained clinically silent, despite a large number (51%) with previous arterial events or arterial lesions at molecular diagnosis. Patients treated with celiprolol had a better survival than others (p = 0.0004). The observed reduction in mortality was dose-dependent: the best protection was observed at the dose of 400 mg/day versus <400 mg/day (p = 0.003).

The study design has its limitations, so it is therfore not possible to definitively conclude that celiprolol influenced the positive outcome reported. However, when the treated and untreated groups are compared there does seem to be a significant difference in survival. Future randomized prospective trials that compare celiprolol to another beta-blocker in molecularly confirmed vEDS patients are therefore needed in order to know the true effects of this medication and its benefits in this population.

Read the full article here

Read an editorial on this paper, co-authored by Prof Julie De Backer (HTAD-WG Chair and MSA-WG member), entitled Vascular Ehlers-Danlos Syndrome Management: The Paris Way, A Step Forward on a Long Road, here

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