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Although conjugated equine estrogen (CEE) is a natural estrogen, it is not pre-

ferred for the induction of puberty because it is a mixture of more than 100

types of estrogens, with estrone being the predominant form, rather than estra-

diol. Use of ethinyl estradiol is associated with higher incidence of cardiovas-

cular events, hypertension, and thromboembolism and hence should be avoided

for induction of puberty.

67. What are the fertility prospects in patients with TS?

Although pregnancy is rare in TS, fertility prospects in TS can be improved

either by cryopreservation of ovarian tissue or donor oocyte followed by

in vitro fertilization and embryo transfer. Cryopreservation of ovarian tissue

should be performed during prepubertal period and is preferred in patients

with mosaicism. Donor oocyte may be the only fertility option for majority of

those with classical TS and pregnancy outcomes are similar to non-TS women

who have undergone embryo transfer after oocyte donation.

68. What are the contraindications to pregnancy in patients with TS?

Presence of cardiovascular anomalies like aortic dilatation, bicuspid aortic valve

and hypertension, or past history of surgery for cardiac defects increases the risk

of aortic dilatation and dissection during pregnancy and should be considered as

relative contraindications for pregnancy. However, ASI >2 cm/m^2 is an absolute

contraindication to pregnancy. Therefore, thorough cardiac evaluation including

cardiac MRI is mandatory in women with TS planning pregnancy.

69. What are the pregnancy outcomes in women with TS?

Although most women with TS are infertile, spontaneous pregnancy can occur in

2–3 % of these patients and most of these women have mosaicism. Pregnancy in

women with TS is associated with increased incidence of miscarriages, stillbirths,

and congenital malformations. High incidence of adverse pregnancy outcomes in

TS is attributed to defective oocyte, increased probability of chromosomal abnor-

malities in the fetus, and decreased endometrial receptivity. In addition, due to

their small pelvic size, most women with TS require cesarean section.

70. What are the causes of mortality in Turner syndrome?

Mortality in women with Turner syndrome is threefold higher than in the gen-

eral population. Cardiovascular disorders, including coronary artery disease,

cerebrovascular disease, aortic aneurysm, and congenital heart disease are the

most common causes of mortality and contribute to 41 % of excess mortality in

TS. Pneumonia, recurrent urinary tract infection with renal dysfunction, and

diabetes also lead to excess mortality in women with TS. Mortality is higher in

those with classical TS (SMR 4.4) as compared to those with mosaic TS (SMR

2.2), and this is due to aortic aneurysm and cardiovascular congenital

anomalies.

8 Turner Syndrome