Do 10-25% of those with Classical EDS who tested negative for Vascular EDS have vascular issues?

I am not aware of an absolute increased risk for a "vascular issue" in the other forms of EDS. One "vascular concern" that has been evaluated in individuals with classical EDS and hypermobility EDS is the issue of aortic diameter. After identifying a small number of individuals with classical EDS who have a larger than normal aortic diameter, researchers in Cincinnati and the NIH evaluated the aorta of 72 individuals with EDS and found aortic root dilation (ARD) in 20 individuals; 14 of 42 with classical EDS and 6 of 29 with hypermobile EDS. Any aortic diameter greater than 2 standard deviations beyond the mean (95 percentile) was recorded as "dilated". Now, as only one ultrasound evaluation was done on each patient, we do not know if and how the size of the aorta might change with time in EDS individuals. Longitudinal studies are necessary to answer this question. [Wenstrup RJ et al. “Prevalence of aortic root dilation in the Ehlers-Danlos syndrome.” Genet Med. 2002; 4(3): 112–7] Answered by Melanie Pepin, MS,CGC

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