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reference materials

Understanding Vascular Complications: A Primer of Essential Definitions

key information

source: Ehlers-Danlos National Foundation (EDNF)

year: 2009

authors: James H. Black III, George Arnaoutakis, Jennifer Fairman


Blood vessels derive their strength from a web of structural proteins. The vascular complications of EDS arise from a genetic protein defect that predisposes blood vessel walls to be weak. True aneurysms occur in arteries and are defined as a dilatation of the blood vessel wall, but with all three layers intact. There is a special physics law which states that more stress is exerted on larger diameter blood vessels. This explains why as the diameter of arterial aneurysms increases, they are more prone to rupture, in general. However, in EDS aneurysm rupture is unpredictable and may occur at any diameter. Aneurysm rupture is a life threatening condition, but fortunately true aneurysm formation is a relatively rare phenomenon in EDS, occurring in approximately 15% of patients. Aneurysms may be asymptomatic, or they may cause pain in the region of the aneurysm.

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