This cohort includes patients with molecularly proven vEDS followed between January 2000 and March 2017 in the French National Reference Center for rare vascular diseases at Hôpital Européen Georges Pompidou (Paris, France). The secondary objective was the assessment of brain ischemia secondary to SAT dissection in this specific population and the search for associated factors. Our primary objective was to determine the prevalence and cumulative incidence of all arterial lesions involving SAT in patients with vEDS with a longitudinal follow-up. 5–9 However, global prevalence of SAT dissections in patients with vEDS is probably underestimated because of the lack of systematic cervical arterial imaging and the absence of longitudinal follow-up in previous studies. About 25% of all arterial complications involve head and neck vessels 4 and supra-aortic trunks (SAT) dissection seems as a typical and frequent cerebrovascular complication in patients with vEDS with prevalence varying from 9.4% to 17% in ancient series. 3Īrterial complications in vEDS can affect all anatomic areas, with rupture or dissection of large and medium-sized arteries. Vascular EDS diagnosis is clinically suggested by major and minor criteria and requires molecular confirmation. 2 Collagen type III deficit can lead to life-threatening complications because of arterial, intestinal, and/or uterine fragility. 1 vEDS is because of pathogenic variants in the CO元A1 gene encoding for procollagen type III and de novo variants occur in roughly 50% of the cases. 130050) is a rare autosomal dominant connective tissue disorder estimated to affect 1 in 50 to 200 000 individuals. Vascular Ehlers-Danlos syndrome (vEDS, OMIM No. Customer Service and Ordering Information.Stroke: Vascular and Interventional Neurology.Journal of the American Heart Association (JAHA).Circ: Cardiovascular Quality & Outcomes.Arteriosclerosis, Thrombosis, and Vascular Biology (ATVB).
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