Incidence of Aortic Dissection in Turner Syndrome

The complexity of fertility preservation for women with Turner syndrome and the potential risks of pregnancy and cardiovascular complications

“…In some cases, adult women receive a diagnosis of Turner syndrome is a life-threatening catastrophe. 6 In line with international guidelines, medical assistance to become pregnant is not recommended if congenital heart and/or vessel abnormalities, operated or not, or acquired aortic dilation is present in women with Turner syndrome. 5,7 Other options for parenthood should be advised.…”

“…11 The most recent survey of women with Turner syndrome in Sweden, with a follow-up of 23 years according to the national guidelines, reported on 198 women, 9 of whom suffered from aortic dissection, at a mean age of 34 years. 6 The majority of these patients had congenital bicuspid aortic valve or coarctatio aortae. With this in mind, if these cardiac features are confirmed early in life, it is advisable not to encourage fertility preservation during childhood or future pregnancies.…”

“…Importantly, the majority of women with aortic dissection had a mixed genotype and only a minority had a monosomy. 6 Hence, genotype cannot be used for cardiovascular risk evaluation. It is gratefully acknowledged that…”

“…If aorta dilation in relation to body surface area is confirmed, more intensive lowering of blood pressure and consultations with a cardiologist are initiated for possible surgery. The congenital aberrations (bicuspid aortic valve, coarctatio of the aortae) and/or aortic dilation are strongly associated with future aortic dissection, which is a life‐threatening catastrophe 6 . In line with international guidelines, medical assistance to become pregnant is not recommended if congenital heart and/or vessel abnormalities, operated or not, or acquired aortic dilation is present in women with Turner syndrome 5,7 .…”

“…The most recent survey of women with Turner syndrome in Sweden, with a follow‐up of 23 years according to the national guidelines, reported on 198 women, 9 of whom suffered from aortic dissection, at a mean age of 34 years 6 . The majority of these patients had congenital bicuspid aortic valve or coarctatio aortae.…”