Objective To determine the characteristics and outcomes of pregnancy in women with Turner syndrome. Design Retrospective 20‐year cohort study (2000–20). Setting Sixteen tertiary referral maternity units in the UK. Population or sample A total of 81 women with Turner syndrome who became pregnant. Methods Retrospective chart analysis. Main outcome measures Mode of conception, pregnancy outcomes. Results We obtained data on 127 pregnancies in 81 women with a Turner phenotype. All non‐spontaneous pregnancies (54/127; 42.5%) were by egg donation. Only 9/31 (29%) pregnancies in women with karyotype 45,X were spontaneous, compared with 53/66 (80.3%) pregnancies in women with mosaic karyotype 45,X/46,XX (P < 0.0001). Women with mosaic karyotype 45,X/46,XX were younger at first pregnancy by 5.5–8.5 years compared with other Turner syndrome karyotype groups (P < 0.001), and more likely to have a spontaneous menarche (75.8% versus 50% or less, P = 0.008). There were 17 miscarriages, three terminations of pregnancy, two stillbirths and 105 live births. Two women had aortic dissection (2.5%); both were 45,X karyotype with bicuspid aortic valves and ovum donation pregnancies, one died. Another woman had an aortic root replacement within 6 months of delivery. Ten of 106 (9.4%) births with gestational age data were preterm and 22/96 (22.9%) singleton infants with birthweight/gestational age data weighed less than the tenth centile. The caesarean section rate was 72/107 (67.3%). In only 73/127 (57.4%) pregnancies was there documentation of cardiovascular imaging within the 24 months before conceiving. Conclusions Pregnancy in women with Turner syndrome is associated with major maternal cardiovascular risks; these women deserve thorough cardiovascular assessment and counselling before assisted or spontaneous pregnancy managed by a specialist team. Tweetable abstract Pregnancy in women with Turner syndrome is associated with an increased risk of aortic dissection.
Objective: Direct current cardioversion (DCCV) in pregnancy is rarely required and typically only documented in single case reports or case series. A recent UK confidential enquiry reported on several maternal deaths where appropriate DCCV appeared to have been withheld. Design: Retrospective cohort study. Setting: Seventeen UK and Ireland specialist maternity centres. Sample: Twenty-seven pregnant women requiring DCCV in pregnancy. Main outcome measures: Maternal and fetal outcomes following DCCV. Results: Twenty-seven women had a total of 29 DCCVs in pregnancy. Of these, 19 (70%) initial presentations were to Emergency Departments and eight (30%) to maternity settings. There were no maternal deaths. Seventeen of the women (63%) had a prior history of heart disease. Median gestation at DCCV was 28 weeks, median gestation at delivery was 35 weeks, with a live birth in all cases. The abnormal heart rhythms documented at the first cardioversion were atrial fibrillation in 12/27 (44%) cases, atrial flutter in 8/27 (30%), supraventricular tachycardia in 5/27 (19%) and atrial tachycardia in 2/27 (7%). Fetal monitoring was undertaken following DCCV on 14/29 (48%) occasions (10 of 19 (53%) at ≥26 weeks) and on 2/29 (7%) occasions, urgent delivery was required post DCCV. Conclusions: Direct current cardioversion in pregnancy is rarely required but should be undertaken when clinically indicated according to standard algorithms to optimise maternal wellbeing. Once the woman is stable post DCCV, gestation-relevant fetal monitoring should be undertaken. Maternity units should develop multidisciplinary processes to ensure pregnant women receive the same standard of care as their non-pregnant counterparts.
While most breech babies are delivered by caesarean section, a small number are born vaginally. Detailed counselling and an accoucher skilled in vaginal breech birth (VBB) are essential for offering the modality. External cephalic version (ECV) is safe, acceptable to most women, has few contraindications and increases vaginal birth rates. Undiagnosed term breech presentation can largely be prevented by routine third-trimester ultrasound.Research is needed to evaluate the efficacy of VBB simulation training in clinical practice. Learning objectivesTo understand the evidence base around breech presentation at term, including ECV, VBB, caesarean section and adjunctive methods of cephalic version. To understand the prerequisites for, and contraindications to, VBB.To understand the principles of physiological breech birth.To appreciate the variation in management of term breech presentation internationally and understand the impact of various publications. Ethical issuesWith falling rates of VBB, and many units now unable to offer the modality, questions surround the appropriateness of national guidance continuing to present vaginal breech as routine. Conversations around breech birth isolate discussion of risk to the fetus. Maternal risk, which may extend beyond the current pregnancy, cannot be overlooked.
Key content Pregnancy‐induced changes in haemodynamic physiology can place considerable strain on cardiac function in some women with valvular disease. Regurgitant valve lesions are usually better tolerated in pregnancy than stenotic lesions, although the risk of obstetric complications is increased in both. Pre‐conception counselling is essential for all women with valvular disease. Optimising anticoagulation is a particular challenge in women with mechanical valves. Learning objectives To understand the World Health Organization (WHO) classification of maternal cardiac disease and how this affects counselling regarding decisions around pregnancy for women with valve pathology. To understand how valve disease affects pregnancy and vice versa. To gain insight into the anaesthetic and haematological considerations for managing women with valvular disease. Ethical issues What is the optimum anticoagulation regimen for women with mechanical heart valves in pregnancy that balances both maternal and fetal risks?
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