Prompt multidisciplinary surgical management of aortic dissection in a pregnant patient with Ehlers-Danlos syndrome can achieve maternal and fetal survival, though long-term staged aortic replacement may be necessary.
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May support prompt surgery for aortic dissection in pregnant EDS; leaves open standardized long-term surveillance protocols.
Pregnancy complicated by aortic dissection in patients with hereditary disorder of connective tissue presents interesting considerations including management of caesarean section with the unexpected need for cardiac surgery in emergency. Generalizations can be made on management principles with long-term follow-up requiring an aggressive individualized approach by a multidisciplinary team. A 33-year-old parturient presenting an aortic dissection at 37 weeks gestation required prompt diagnosis 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 transverse aortic arch and thoracoabdominal aortic replacement.
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Gérard Babatasi (1997) studied this question.
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