EhlerseDanlos syndrome
Type IV EhlerseDanlos syndrome is associated with aortic involvement as well as bleeding and poor
wound healing. Due to the tissue fragility, patients are at a risk of aortic dissection even with a normalsize
aorta. The risks of surgery are greater than for other forms of connective tissue disease, and therefore prophylactic aortic root replacement is not indicated. Pregnancy is associated with an
increased risk of aortic dissection as well as uterine rupture, and therefore pregnancy is not advised in
these patients