Pippa Kyle

Myasthenia in pregnancy: best practice guidelines from a UK multispecialty working group

A national UK workshop to discuss practical clinical management issues related to pregnancy in women with myasthenia gravis was held in May 2011. The purpose was to develop recommendations to guide general neurologists and obstetricians and facilitate best practice before, during and after pregnancy. The main conclusions were (1) planning should be instituted well in advance of any potential pregnancy to allow time for myasthenic status and drug optimisation; (2) multidisciplinary liaison through the involvement of relevant specialists should occur throughout pregnancy, during delivery and in the neonatal period; (3) provided that their myasthenia is under good control before pregnancy, the majority of women can be reassured that it will remain stable throughout pregnancy and the postpartum months; (4) spontaneous vaginal delivery should be the aim and actively encouraged; (5) those with severe myasthenic weakness need careful, multidisciplinary management with prompt access to specialist advice and facilities; (6) newborn babies born to myasthenic mothers are at risk of transient myasthenic weakness, even if the mothers myasthenia is well-controlled, and should have rapid access to neonatal high-dependency support.

Foetal congenital heart disease: obstetric management and time to first cardiac intervention in babies delivered at a tertiary centre.

OBJECTIVES The aim of this study was to determine the timing of neonatal cardiac intervention in babies with antenatally diagnosed congenital heart disease and the impact on obstetric management. METHODS A retrospective review of all deliveries between January, 2008 and December, 2009 was conducted in a tertiary centre with foetal and paediatric cardiology, maternal-foetal medicine, and obstetric units. All live births with antenatally detected congenital heart disease were included. Data were collected from foetal, paediatric cardiology, and maternity databases and records. Induction, delivery mode, and timing of the first cardiac intervention in the neonate were studied. RESULTS 205 deliveries were included. Induction and elective Caesarean section rates were 51.2% (105/205) and 14.1% (29/205), respectively. The vaginal delivery rate was 56% (115/205). There was a non-significant trend towards a higher rate of vaginal delivery after spontaneous labour than after induction (75% versus 66%; p = 0.234). The rate of neonatal cardiac intervention during the initial stay was 59.5% (122/205); it was 18.5% (38/205) within 48 hours and 25.8% (53/205) within 72 hours. The median time to first intervention was 4 days (interquartile range 2-8). Babies with hypoplastic left heart syndrome (median 3, interquartile range 2-6), transposition of the great arteries (median 1, interquartile range 0-4.5), and arrhythmia (median 0.5, interquartile range 0-1) had a significantly earlier time to first intervention compared with those with other conditions (p = 0.001). CONCLUSION Vaginal delivery can be achieved in women delivering babies with major congenital heart disease at a tertiary centre. Delivery in or near a tertiary centre is recommended for patients requiring early intervention, of which many can be identified in advance.

Survival of pregnancies with small for gestational age detected before 24 weeks gestation

OBJECTIVE Counselling women where severe growth abnormalities are detected early in the pregnancy is often difficult due to a paucity of outcome data of this specific subset of early onset disease. This study therefore aimed to assess the outcome of pregnancies where an estimated fetal weight less than the third centile were detected prior to 24 weeks gestation. STUDY DESIGN A retrospective study in two London teaching hospitals, over an eight year period was performed, analysing all pregnancies with an ultrasound estimated fetal weight less than the third centile prior to 24 weeks gestation. Outcome data: intrauterine death, neonatal death, survival to discharge, gestation at delivery and birthweight were collected. RESULTS Out of 20 pregnancies included in the analysis, six died in utero, two died in the neonatal period and 12 (60%) survived until discharge. Of the livebirths, 67% delivered preterm and 100% percent of livebirths were delivered by Caesarean Section. CONCLUSION When severe growth abnormalities were detected before 24 weeks, more than half of pregnancies resulted in survival to neonatal discharge. There was an increased incidence of preterm delivery, caesarean section and neonatal unit admission. This information is useful in counselling parents.

Third trimester amniocentesis for diagnosis of inherited bleeding disorders prior to delivery

X‐linked and autosomally inherited bleeding disorders confer a risk of foetal intracranial haemorrhage during delivery. Conventional prenatal diagnosis involving chorionic villus sampling or early amniocentesis is primarily aimed at offering the choice of pregnancy termination. Currently, non‐invasive procedures, involving analysis of free foetal DNA in the maternal circulation, are restricted to gender determination, and are of limited value in women at risk of carrying a foetus with a bleeding disorder. These limitations, together with the rising proportion of women shown to be carrying an affected foetus, who decide to continue the pregnancy, have led to the development of prenatal mutation identification via late amniocentesis after 34 weeks of gestation, with the sole aim of directing delivery management. Although this approach has been documented in some cases of potential foetal anomaly, there are no previous reports of its use in women with heritable bleeding disorders. We report a single‐centre experience of this technique in managing nine such deliveries. Of these, three showed an affected foetus, five showed an unaffected foetus and in one case no result could be obtained. In the three affected cases and the one with the inconclusive result restrictive birth plans were implemented, whereas the five unaffected cases underwent routine obstetric management; with one delivery necessitating interventions which would have been contraindicated if foetal status had not been determined. Late amniocentesis is a safe technique for guiding delivery management in women with bleeding disorders where the mutation is known.

Dichorionic triplet pregnancies: risk of miscarriage and severe preterm delivery with fetal reduction versus expectant management. Outcomes of a cohort study and systematic review

In trichorionic pregnancies, fetal reduction from three to two lowers the risk of severe preterm delivery, but provides no advantage in survival. Similar data for dichorionic triamniotic (DCTA) triplets is not readily available.

Screening in the presence of a vanished twin: Nuchal translucency or combined screening test?

We present a case of an initial twin pregnancy with early demise of one twin, at the time of first trimester screening where the addition of biochemical markers helped to detect an aneuploidy in the live fetus. Using the NHS Fetal Anomaly Screening Programme recommendations (NHS FASP, 2010), only nuchal translucency (NT) should have been taken into consideration, which would have given a low risk for the chromosomal abnormalities trisomy 18 and 21. The learning point in this case is that when faced with abnormal biochemistry results in cases with a vanished twin, attention should be paid to the level of the individual biochemical markers.

A descriptive single‐centre experience of the management and outcome of maternal alloantibodies in pregnancy

Haemolytic disease of the fetus and newborn (HDFN) occurs when maternal IgG alloantibodies to fetal red blood cell antigens cross the placenta, causing haemolysis in the fetus and/or neonate. After delivery, the main concern is hyperbilirubinaemia, which can cause neurological damage.

Influence of birthweight on perinatal outcome in fetuses with antenatal diagnosis of congenital heart disease

The incidence of congenital heart disease (CHD) accounts for the largest proportion of infant mortality attributable to birth defects. Associations have previously been reported between CHD and low birthweight. Low birthweight is independently associated with adverse outcome and has characteristically been calculated using population‐based charts. This aim of this study was to determine the incidence of small for gestational age (SGA) in fetuses with CHD utilizing customized birthweight centiles and to determine the effect of SGA on adverse outcome.

The value of obstetric surveillance systems within the National Health Service

There are several obstetric surveillance systems currently used within the National Health Service (NHS). These systems employ a variety of techniques to monitor a range of important health conditions and medical services. Uses of surveillance data include monitoring disease, clinical audit, research and resource planning. A surveillance system must be designed appropriately if it is to achieve its desired purpose and remain a justifiable use of public funds.

PF.43 Low Maternal Serum PAPP-A in the First Trimester and Pregnancy Outcome: An Experience Over 3 Years

Introduction Maternal serum PAPP-A (pregnancy associated plasma protein-A) is a part of combined screening. Previous studies have shown correlation between low PAPP-A and adverse pregnancy outcome. Objective The aim of this study is to establish the positive predictive value of low-PAPP-A in prediction of adverse pregnancy outcomes - pre-eclampsia (PET), pregnancy induced hypertension (PIH), delivery of small for gestational age neonates (SGA) and late pregnancy losses. Materials and Methods 16690 women underwent combined screening from 1/8/2008 to 1/8/2011. 326 women with low PAPP-A (=<0.3 MoM) were identified (1.95%). The median PAPP-A of the screening population was 1.074 MoM. Within this group of pregnancy with low PAPP-A, maternal serum PAPP-A was compared between the subgroups of adverse pregnancy outcome and normal-outcome. Results Abstract PF.43 Table Cases N PAPP-A MoM (Median) p PPV % Normal outcome 146 0.255 - - Adverse outcome 95 0.225 0.004* 39.4 PET 25 0.234 0.184 10.8 PIH 10 0.243 0.769 4.1 SGA 44 0.220 0.004* 21.4 Late pregnancy losses 15 0.191 0.024* 6.6 * Adjusted significance level P < 0.0125 - post hoc Bonferroni correction Conclusion In our screening population, median PAPP-A MoM was higher compared to some previous studies. Maternal serum PAPP-A in pregnancies with adverse outcome was significantly lower than those that resulted in a normal outcome. Compared to the pregnancies with low-PAPP-A but normal outcome, median PAPP-A MoM was significantly lower in pregnancies ending in delivery of small-for-gestational age neonate (customised BW < 10th-centile), and showed a trend towards a decrease in those ending in late-pregnancy losses (>24 weeks).