Endocrine Abstracts

Aims: Women with Turner’s syndrome (TS) are increasingly undertaking pregnancies, either via natural conception (mosaic TS) or assisted conception (AC). Increased TS pregnancies have led to reports suggesting high risk of pregnancy associated aortic dissection (AOD) 2%, and maternal mortality 2% due to underlying aortic valve abnormalities and aortopathies. However, the literature is limited to small case series. We report our practice providing risk-assessment and combined specialist TS endocrinology, cardiology and obstetrics services, resulting in low maternal and fetal complications.

Methods: Data were retrospectively analysed for patients with spontaneous pregnancy or AC with multidisciplinary risk-assessment and care during pregnancy and peurperium, and follow-up in a specialist TS clinic between 2009 and 2016.

Results: Of 87 TS patients, thirteen (15%) had spontaneous pregnancies and six (7%) had AC with three successful pregnancies. Pre-conception risk-assessment and counselling were conducted by a multidisciplinary team comprising TS endocrinologist, obstetrician and cardiologist with echocardiography ± cardiac magnetic resonance imaging. Five patients had pre-existing cardiovascular disease (bicuspid aortic valve [n=5]; dilated aortic root or ascending aorta [n=4]; moderate aortic stenosis [n=1]; hypertension [n=3]; surgery for dilated ascending aorta [n=1]; and for complex congenital heart disease including coarctation [n=1]). During pregnancy, patients underwent regular review by a TS endocrinologist, cardiologist with echocardiography and obstetrician in a high-risk maternity clinic. Fetal cardiologist assessment occurred at 20 weeks gestation. Maternal echocardiography was performed post-partum. There were 16 successful live-births (n=13 spontaneous pregnancies, n=3 AC). One patient developed gestational hypertension which responded to medical therapy. One patient developed mild progression of aortic root dilatation post-partum. There were no cases of moderate or severe aortic dilatation, AOD, pre-eclampsia, maternal deaths or fetal cardiac disease.

Conclusions: Pre-pregnancy risk-assessment and close multidisciplinary care by endocrinology, cardiology and obstetrics services during pregnancy and the peurperium ensures low maternal complications and excellent survival in TS.