PREGNANCY WITH AORTIC DISSECTION IN EHLER-DANLOS-SYNDROME - STAGED REPLACEMENT OF THE TOTAL AORTA (10-YEAR FOLLOW-UP)

Pregnancy complicated by aortic dissection in patients with hereditary disorder of connective tissue presents interesting considerations inc luding management of caesarean section with the unexpected need for ca rdiac surgery in emergency. Generalizations can be made on managment p rinciples with long-term follow-up requiring an aggressive individuali zed approach by a multidisciplinary team. A 33-year-old parturient pre senting an aortic dissection at 37 weeks gestation required prompt dia gnosis of Ehlers-Danlos syndrome in combination with correct surgical therapy resulted in the survival of both the mother and infant. During the 10-year follow-up, multiple complex dissection required transvers e aortic arch and thoracoabdominal aortic replacement. (C) 1997 Elsevi er Science B.V.