Learning Cardiology

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