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Vascular Ehlers Danlos syndrome

vEDS:
- Group of clinically and genetically heterogeneous disorders
- Also known as Type IV EDS
- Prevalence 1 in 250000
- Autosomal dominant
- Mutation in COL3A1 - type III collagen alpha chain 1
- Characterised by aneurysm, dissection or rupture of medium-sized abdominal arteries and abdominal aorta
- No predisposition for aortic root involvement
- most patients will have one complication by the age of 40 years
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