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Our experience with the management of two patients with life-threatening aortic disease during pregnancy is presented with a review of the literature. One of our patients had intimal disruption caused by trauma; the other had probable Ehlers-Danlos type IV syndrome, causing an acute dissection of the descending thoracic aorta and eventually requiring replacement of the aorta from the left subclavian artery to common iliac arteries. The challenge of treating both the pregnant woman and the fetus was managed successfully by an emergent cesarean section followed by Dacron graft replacement of the descending thoracic aorta. The literature reviewed disclosed that aneurysm expansion producing symptoms and dissection is most common during the third trimester and during labor and delivery in patients with or without Marfan's syndrome. Half of the aortic dissections in women less than 40 years of age occur in association with pregnancy. The available evidence indicates that patients with known valvular or aortic disease should have surgical repairs during the first or second trimester and thereafter have delivery by cesarean section. However, patients with acute aortic problems near term appear to be better managed by cesarean section followed promptly by treatment of the aortic disease.

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