Endocrine Abstracts

Aims: Turner’s syndrome (TS) is associated with bicuspid aortic valve (BAV), ascending aortic dilatation (AD), aortic coarctation, and hypertension. Pregnancy in TS is associated with increased risk of aortic dissection (2%), gestational hypertension, pre-eclampsia and a 2% risk of maternal mortality.

This retrospective study aimed to assess the effectiveness of a multidisciplinary team (MDT) comprising endocrinologist, cardiologist and maternal medicine obstetrician providing risk-assessment, pre-conception counselling and close follow-up, on safety and improving pregnancy outcomes in TS.

Methods: From a total of 104 women attending our dedicated TS clinic, we identified 23 spontaneous pregnancies (SP, 3/23 45XO) and six pregnancies with oocyte donation (OD). Clinical data, blood pressure measurements and aortic dimensions (indexed for body surface area) were analysed. Echocardiography, cardiac magnetic resonance (CMR), pre-conception counselling and risk-assessment were performed. Surveillance echocardiography and cardiologist review were performed each trimester and monthly if AD was detected. Echocardiography and CMR were repeated six months post-delivery.

Results: Total 26 successful pregnancies (3 following OD), maternal age 28±7 years. Pre-conception cardiovascular risk-assessment identified hypertension in 6 women, BAV in 7 (1 with moderate aortic stenosis), AD (>2.0 cm/m²) in 4, coarctation repair and correction of congenital heart disease in 1 and thoracic aortic graft in 1. Seven women developed mild aortic dilatation (1±2 mm). Aortic dimensions increased slightly after pregnancy: aortic root 1.79±0.32 cm/m² (1.84±0.37 cm/m²; P=0.07) and ascending aorta 1.69±0.40 cm/m² (1.71±0.41 cm/m²; P=0.83).

Complications were low: gestational hypertension (n=1), diabetes (n=1), aortic dissection (n=0), pre-eclampsia (n=0), and no mortalities. Delivery was at 39±1 weeks, birth weight 3.1±0.6 kg, with 81% caesarian deliveries, n=1 child with TS and n=1 stillbirth.

Conclusions: Pre-conception counselling including risk-assessment by a dedicated MDT, along with close surveillance by a cardiologist with serial echocardiography, ensures low complications and excellent maternal and fetal outcomes, suggesting a more optimistic approach to pregnancy is appropriate in TS women.