Cardiovascular Outcomes of Pregnancy in Turner Syndrome: A Multi-Center Retrospective Case-Control Study

Contact:  Jasmine Grewal, Vancouver, BC

Funding:  Rare Disease Foundation, Canada

Background:  Many women with Turner Syndrome (TS) are keen to become pregnant. A small percentage can become spontaneously pregnant and these women usually have Turner’s mosaicism. With advances in assisted reproductive technology (ART) and oocyte donation, an increasing number of women with TS are becoming pregnant. However, the body of evidence suggests that pregnancy in TS is high risk secondary to aortic dissection and rupture and an increased risk of developing a hypertensive disorder of pregnancy. The maternal mortality rate in TS is estimated to be 2%, 100 fold greater than all-cause mortality during pregnancy in the general population. Women with TS are routinely being referred for pre-conception counselling prior to undergoing ART, unfortunately there is limited data with which to clearly inform women and their care providers of the true risk and risk factors of pregnancy. Consensus group opinions from national societies state that TS itself is a relative contraindication to pregnancy and that any significant cardiac abnormality represents an absolute contraindication. It is generally accepted that aortic dilation (ASI > 2 cm/m2), the presence or prior repair of coarctation of the aorta, and bicuspid aortic valve are risk factors for dissection and rupture in TS. However, there are several unanswered questions including the relative risk of other cardiac lesions and the risk of pregnancy in TS patients without structural heart disease.

 

Objectives

  1. To determine the rate of cardiovascular events in a Turner’s syndrome pregnancy in women with a structurally normal heart.
  2. To compare the rates of cardiovascular events in a Turner’s syndrome pregnancy between women with and without a structurally normal heart.
  3. To compare the rate of cardiovascular events between pregnant TS women with a structurally normal heart and non-pregnant TS women with a structurally normal heart.