Pregnancies in women with Turner syndrome: a retrospective multicentre UK study.

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.

Seasonal and SARS-CoV-2 pandemic changes in the incidence of gestational diabetes.

OBJECTIVE: To test the hypothesis that there is seasonal variation in the rates of gestational diabetes (GDM) diagnosed using a 2-hour oral glucose tolerance test. DESIGN: Monthly assessment of the percentage of women screened from 1 April 2016 to the 31 December 2020 who were diagnosed as having gestational diabetes. SETTING: London teaching hospital. POPULATION: 28 128 women receiving antenatal care between 1 April 2016 and 31 December 2020. METHODS: Retrospective study of prospectively collected data. MAIN OUTCOME MEASURES: Proportion of women screened diagnosed as having gestational diabetes. RESULTS: The mean (SD) percentage of women diagnosed with GDM was 14.78 (2.24) in summer (June, July, August) compared with 11.23 (1.62) in winter (P < 0.001), 12.13 (1.94) in spring (P = 0.002) and 11.88 (2.67) in autumn (P = 0.003). There was a highly significant positive correlation of the percentage testing positive for GDM with the mean maximum monthly temperature (R2  = 0.248, P < 0.001). There was a statistically significant 33.8% increase in the proportion of GDM diagnoses from June 2020 onwards, possibly related to a reduction in exercise secondary to the Covid-19 pandemic. CONCLUSIONS: There is a 23.3% higher rate of GDM diagnoses in the warmer summer months. There has been a 33.8% rise in GDM diagnoses associated with the Covid-19 pandemic. TWEETABLE ABSTRACT: Rates of GDM are higher in summer and since the onset of the Covid-19 pandemic.

Impact of changes to national UK Guidance on testing for gestational diabetes screening during a pandemic: a single-centre observational study.

OBJECTIVE: To examine the differences in detection rate for gestational diabetes (GDM) comparing the methodology recommended by the National Institute for Health and Clinical Excellence (NICE) compared with testing described as appropriate during the Covid-19 pandemic by the Royal College of Obstetricians and Gynaecologists (RCOG). DESIGN: Cohort study of women delivering between 1 January 2016 and 1 July 2020. SETTING: London Teaching Hospital. POPULATION: All women delivering between 1 January 2016 and 13 May 2020 and follow up of women screening negative between 1 April 2020 and 13 May 2020. METHODS: Retrospective study of prospectively collected data. MAIN OUTCOME MEASURES: Detection rate of gestational diabetes. RESULTS: Using the RCOG guidance, the overall rate of women identified as having gestational diabetes fell from 7.7% (1853/24168) to 4.2% (35/831)(P = 0.0003). Of 230 women who tested negative according to the RCOG criteria from 1 April to 13 May but who subsequently had an oral glucose tolerance test, 47 (20.4%) were diagnosed as having gestational diabetes according to the NICE criteria. CONCLUSIONS: In our setting, the RCOG Covid-19 gestational diabetes screening regime failed to detect 47 of 82 (57%) women subsequently identified as gestational diabetics, and therefore cannot be recommended for general use. TWEETABLE ABSTRACT: Screening for GDM using RCOG Covid criteria reduced detection rates.

Pregnancy outcomes in women with primary biliary cholangitis and primary sclerosing cholangitis: a retrospective cohort study.

OBJECTIVE: To determine maternal, obstetric and neonatal outcomes in a cohort of women with primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). DESIGN: Retrospective cohort study. SETTING: Ten specialist centres managing pregnant women with liver disease. POPULATION: Women with a diagnosis of PBC and PSC and a pregnancy of ≥20 completed weeks of gestation. METHODS: Retrospective case notes review. MAIN OUTCOME MEASURES: Adverse outcomes were defined as: maternal - development of ascites, variceal bleeding, encephalopathy and jaundice; obstetric events - gestational hypertension, pre-eclampsia and postpartum haemorrhage; and neonatal - stillbirth, preterm delivery and admission to neonatal unit. The relationship of alanine transferase (ALT) and bile acid levels with gestation at delivery was studied. RESULTS: The first recorded pregnancies of 34 women with PSC and 27 women with PBC were analysed. There were 60 live births and one intrapartum stillbirth that did not occur in the context of maternal cholestasis. The overall median gestation of delivery was 38 weeks but the rate of preterm birth was 28% (17/61 deliveries), 76% (13/17) of which were spontaneous. Gestation at birth negatively correlated with maternal serum ALT concentration at booking (P = 0.017) and serum bile acid concentration during pregnancy (P = 0.016). There were no other significant correlations and maternal and neonatal outcomes were good. CONCLUSIONS: Pregnancy in PBC and PSC is well tolerated, but women should be counselled regarding the increased risk of preterm birth. Measurement of maternal ALT and bile acids may help identify women at risk of preterm delivery. TWEETABLE ABSTRACT: Pregnancy in women with PBC and PSC is well tolerated; however, rates of preterm birth are high and are related to maternal bile acid levels.

Maternal and fetal outcomes in pregnancies complicated by the inherited aortopathy Loeys-Dietz syndrome.

OBJECTIVE: Pregnancies in women with Loeys-Dietz syndrome (LDS) are rare and are typically documented in case reports only. Early reports suggested high rates of maternal complications during pregnancy and the puerperium, including aortic dissection and uterine rupture, but information on fetal outcomes was very limited. DESIGN: A retrospective cohort study. SETTING: Eight specialist UK centres. SAMPLE: Pregnant women with LDS. METHODS: Data was collated on cardiac, obstetric, and neonatal outcomes. MAIN OUTCOME MEASURES: Maternal and perinatal outcomes in pregnancies complicated by LDS. RESULTS: Twenty pregnancies in 13 women with LDS were identified. There was one miscarriage, one termination of pregnancy, and 18 livebirths. In eight women the diagnosis was known prior to pregnancy but only one woman had preconception counselling. In four women the diagnosis was made during pregnancy through positive genotyping, and the other was diagnosed following delivery. Five women had a family history of aortic dissection. There were no aortic dissections in our cohort during pregnancy or postpartum. Obstetric complications were common, including postpartum haemorrhage (33%) and preterm delivery (50%). In all, 14/18 (78%) of deliveries were by elective caesarean section, at a median gestational age at delivery of 37 weeks. Over half the infants (56%) were admitted to the neonatal unit following delivery. CONCLUSION: Women with LDS require multidisciplinary specialist management throughout pregnancy. Women should be referred for preconception counselling to make informed decisions around pregnancy risk and outcomes. Early elective preterm delivery needs to be balanced against a high infant admission rate to the neonatal unit. TWEETABLE ABSTRACT: Pregnancy outcomes in women with Loeys-Dietz syndrome.

Anaesthetic management of pregnant patients with cardiac implantable electronic devices: case reports and review.

Heart disease is a leading cause of maternal mortality and morbidity. Pregnant women with structural, conduction or degenerative cardiac disease who require rhythm control or who are at high risk of sudden cardiac death may carry a cardiac implantable electronic device or may occasionally require the insertion of one during their pregnancy. These women are now encountered more frequently in clinical practice, and it is essential that a multidisciplinary approach, beginning from the early antenatal phase, be adopted in their counselling and management. Contemporary cardiac rhythm control devices are a constantly evolving technology with increasingly sophisticated features; anaesthetists should therefore have an adequate understanding of the principles of their operation and the special considerations for their use, in order to enable their safe management in the peripartum period. Of particular importance is the potential adverse effect of electromagnetic interference, which may cause device malfunction or damage, and the precautions required to reduce this risk. The ultimate goal in the management of this patient subgroup is to minimise the disruption to cardiovascular physiology that may occur near the time of labour and delivery and to control the factors that impact on device integrity and function. We present the ante- and peripartum management of two pregnant women with an implantable cardioverter-defibrillator, followed by a review and update of the anaesthetic management of parturients with cardiac implantable electronic devices.

Pre-pregnancy counseling for women with heart disease: A prospective study.

BACKGROUND: Women with cardiac disease and their infants are at a greater risk of mortality and morbidity during pregnancy. Expert groups recommend preconception counseling (PCC) for all women with cardiac disease so they are made aware of these risks. We have run a specialist maternal cardiac clinic since 1996. The aim of this study was to evaluate the experience of women who have received PCC within an established multidisciplinary tertiary clinic and to establish their views regarding the counseling they received. METHODS: Single centre prospective study using a patient questionnaire was given to women attending a specialist cardiac preconception counseling clinic from November 2015 to August 2016, with analysis of descriptive data and free text comments from the questionnaire responders. RESULTS: 40/65 returned patient questionnaires. Prior to the consultation fewer than half felt well informed regarding how their heart disease could impact upon pregnancy but a similar proportion felt nonetheless that they would be able to have a healthy pregnancy. Women reported two main areas of concerns, their own health (whether they would survive a pregnancy) and the health of their child. 15% of women reported that these concerns had prevented them from pursuing a pregnancy. Women reported high satisfaction rates with the clinic. CONCLUSIONS: There is an increasing demand for PCC services for women with cardiac disease; our study is the first attempt to determine both the acceptability and the impact of PCC from the patient perspective. Patients reported a high level of satisfaction with the service provided.

Anaesthetic management of parturients with univentricular congenital heart disease and the Fontan operation.

Women with a single ventricle circulation palliated with the Fontan operation require specialist multidisciplinary management. We report 14 such cases with successful pregnancies and detail the pathophysiology encountered. A combined obstetric and cardiac service between Chelsea and Westminster Hospital and Royal Brompton Hospital provides care for women with heart disease, and maintains a prospective database of referred women. We searched this database for women with a known Fontan circulation and reviewed the case notes and electronic patient records between January 1994 and December 2015. Eight women palliated with the Fontan operation delivered 14 live babies over the study period, with detailed peripartum management available for 11. Low-dose combined spinal-epidural or epidural labour analgesia was the intended mode of analgesia or anaesthesia for all deliveries (depending on clinical scenario and clinician preference), and was performed in 79%. Seven cases (50%) had a caesarean delivery. A neuraxial catheter technique was preferred (86%), whether or not vaginal delivery was attempted first. There were no deliveries under general anaesthesia. Fifty percent of cases were complicated by postpartum haemorrhage. Other peripartum complications included arrhythmias (29%), chest pain (14%) and intrauterine growth restriction (57%). Women with a Fontan circulation are increasingly encountered in obstetric practice. A good understanding of the underlying anatomy and its impact on physiology, coupled with meticulous care are essential to allow safe delivery for mother and baby. Multidisciplinary input into peripartum care is required, with anticipation of increased risk of complications such as haemorrhage and arrhythmias.