Materno-Fetal Cardiovascular Complications in Turner Syndrome after Oocyte Donation: Insufficient Prepregnancy Screening and Pregnancy Follow-Up Are Associated with Poor Outcome

Abstract

OD pregnancies in TS who have not been managed following recent specific recommendations were at high risk for maternal death by aortic dissection and for preeclampsia and its complications (fetal distress and in utero growth retardation). These recommendations include previous echocardiography, thoracic magnetic resonance imaging, and overnight blood pressure monitoring associated with a tight follow-up during pregnancy. Until future assessment of these recent recommendations, pregnancies obtained in TS after OD must be still considered as very high-risk pregnancies.

Cardiac magnetic resonance imaging in Turner syndrome

“…28 Patients with TS with a desire to carry a pregnancy have therefore sought oocyte donation and implantation, which has been reported as successful, albeit with a higher rate of miscarriage. 28 However, several reports have been published of aortic dissection and deaths during and after pregnancy in patients with TS, with an estimated rate of 2%. 10 In this study, however, data were collected by survey, and only half of oocyte donation programs in the United States responded, raising the question of an underestimation of the true dissection rate in pregnancy.…”

“…Two women in this report died as a result of aortic dissection, one noted to have dilatation in the second trimester, with subsequent dissection necessitating delivery at 38 weeks, and the other with documented prepregnancy normal echo, dilatation noted at 6-month gestation, and fatal dissection 7 days after Caesarean section at 38-week gestation. 28 A larger study of 276 TS subjects, who underwent CMR with measurements throughout the aortic arch, reported 5 women who had spontaneous pregnancies, resulting in 7 children, and another 5 women who had 6 successful pregnancies via assisted reproductive technique, resulting in 7 children (including one twin gestation). However, most of the pregnancies reported occurred prior to cardiovascular screening, and thus, CMR data were obtained after pregnancy and compared with age-matched and aortic valve structure matched nulliparous TS controls.…”

Fertility in Turner syndrome

“…These include thyroid dysfunction, obesity, diabetes, hypertension and preeclampsia, which occur in approximately 40% of patients with TS compared with 6-12% of the general population. 2,[29][30][31] Severe complications such as deterioration of congenital heart disease, heart failure, aortic dissection and sudden death are well described. [32][33][34] Importantly, women with TS are at increased risk of development of aortic cystic medial necrosis independent of congenital heart disease; 10% of patients with aortic dilatation, dissection or rupture have no prior cardiac risk factors.…”

“…In adult women with TS, pregnancy is associated with increased risk of maternal mortality, foetal loss, foetal congenital abnormality and abnormal karyotype. [1][2][3] Success rates are low for many invasive reproductive techniques, and newer technologies such as ovarian cryopreservation are presently considered experimental.…”

“…Adult reproductive endocrinologists involved in fertility assistance have relatively recently begun to appreciate the mortality risk to the TS mother that pregnancy may entail. 2,4 Assisted reproduction techniques possible in children prior to the decline of ovarian reserve, such as ovarian tissue cryopreservation, require paediatric surgery. Ethical challenges relating to consent and beneficence thus arise for the paediatric endocrinologist and gynaecologist.…”