How can we make pregnancy safe for women with Turner syndrome?

Abstract

Pregnancy is a crucial issue in patients with Turner syndrome (TS). Although natural pregnancies have been reported in 4-7% of TS patients, most women will need assisted reproductive technologies (ART) with oocyte donation. The main issue is the maternal mortality rate that is higher than in the general population. It is related to cardiovascular anomalies and particularly aortic dissection. Turner syndrome, per se, is not a contraindication for pregnancy, but a multidisciplinary screening is mandatory before initiating a pregnancy. It includes repeated aortic diameters evaluation, blood pressure measurement and biological testing evaluating thyroid and liver functions, as well as blood glucose level. In order to make the pregnancy safe, contraindications of pregnancy should be respected and identification of high-risk patients for cardiovascular events should be performed. Hypertension and pre-eclampsia prevention may benefit from beta-blockers and aspirin, respectively. Collaborations between endocrinologists, cardiologists and obstetricians are mandatory during pregnancy and even in the post-partum period. Counseling the patients about the risks of pregnancy, screening them and spreading the international guidelines to physicians taking care of patients with TS are the three pillars of a safe pregnancy.

Recognition and management of adults with Turner syndrome: From the transition of adolescence through the senior years

“…Care should be taken to ensure that the communication style of the provider matches the receptive language abilities of the patient, a nuance that can be overlooked if the woman has good expressive language. An approach to pregnancy emphasizing the need to coordinate care before, during, and after pregnancy was published recently and includes a checklist of possible examinations (Donadille, Bernard, & Christin‐Maitre, , table 2).…”

Congenital heart defects associated with aneuploidy syndromes: New insights into familiar associations

“…Aortic dissection is a rare but feared complication in this patient population, occurring at greater frequency and significantly younger age than the general population, particularly during pregnancy. Those women attempting pregnancy, typically via assisted reproductive technologies, should be closely monitored with cardiac imaging, before, during, and after pregnancy (Donadille, Bernard, & Christin‐Maitre, ). Congenital coronary artery anomalies are common in Turner syndrome women (20%), and their anatomy should be delineated in the planning of aortic surgery (Viuff et al, ).…”

Should women with Turner syndrome be allowed to carry their own pregnancies?

“…This study included 101 women with TS, two of whom had an aortic dissection, outside of pregnancy (30). The increased cardiovascular demands of pregnancy put women with TS at even greater risk (31). These demands are increased further in a multiple pregnancy with an estimated 5-fold higher risk of maternal mortality compared to a singleton pregnancy with a 110fold increase in risk over the general population of the U.S. of 0.018% in 2014 (32,33).…”

“…In the Bernard et al (5) series of spontaneous pregnancies, there was a 13.3% rate of hypertensive disorders of pregnancy/preeclampsia, which compares to an expected rate of hypertensive disorders of pregnancy/preeclampsia of 2.8% based on the average age of this cohort. They are also at increased risk for liver diseases, gestational diabetes and thyroid dysfunction, spontaneous miscarriage, caesarean section, preterm delivery and low birth weight (5,17,31,46).…”