[Procreation in Turner's syndrome: which recommendations before, during and after pregnancy?]

Turner's Syndrome (TS) is characterized by an ovarian failure which occurs in most cases before puberty and leads to infertility. In less than 10% of women with TS, puberty may occur and spontaneous pregnancies are possible with a high risk of fetal loss, chromosomal and congenital abnormalities. Fertile women with TS should therefore be counselled with regard to these increased risks and be offered prenatal diagnosis testing. For all the other women with TS, in vitro fertilization with oocyte donation (OD) has dramatically transformed the prognosis of infertility. However, in the same time, it has become obvious that pregnancies in TS either spontaneous or obtained after oocyte donation are at very high risk of possible sudden death. Miscarriages are very frequent probably linked to uterine abnormalities. The most specific risks lie in cardiovascular complications involving aortic root dissection, severe hypertension (HTA) or ventricular insufficiency. In fact pregnancies in TS women cumulate the risk of congenital heart defects and HTA associated to TS, the risk of preeclampsia associated to oocyte donation and the increased cardiac work necessary for pregnancy. It is therefore absolutely necessary for all women with TS to undergo a full cardiological assessment before seeking to become pregnant including echocardiography, thoracic magnetic resonance imaging (MRI) to verify aortic root, cardiac valves and left ventricular function, hypertension monitoring and treatment. Single embryo transfer must definitively be considered. Cardiovascular surveillance during pregnancy has to be enhanced especially at the third trimester and during the peripartum period, most women requiring caesarean section for delivery because of cephalopelvic disproportion and/or aortic root dilatation risk.