EHLERS-DANLOS-DISEASE PRESENTING DURING P REGNANCY WITH DISSECTION OFTHE AORTA - A CASE-REPORT

Dissection of the aorta is a serious condition but rare in young women , and occurring during the 3rd trimester of pregnancy. The main risk f actors are hypertension and diseases of the connective tissue or of co llagen (Marfan's syndrome and Ehlers-Danlos disease). The authors repo rt a case of dissection of the aorta managed in a pluridisciplinary ma nner by the anaesthetists, cardiologists, obstetricians and cardiothor acic surgeons, which resulted in a favourable outcome for both mother and baby. The diagnosis of Ehlers-Danlos disease was made from the ons et and, over a period of 10 years with CT scan and annual echocardiogr aphic follow-up, total replacement of the supra-coronary aorta was per formed in several stages.