The role of echocardiography in amniotic fluid embolism: a case series and review of the literature.

PURPOSE: Amniotic fluid embolism (AFE) is a rare, but often fatal condition characterized by sudden hemodynamic instability and coagulopathy occurring during labour or in the early postpartum period. As the mechanisms leading to shock and the cardiovascular effects of AFE are incompletely understood, the purpose of this case series is to describe how AFE presents on echocardiography and review limited reports in the literature. CLINICAL FEATURES: We describe three cases of AFE at the Jewish General Hospital, a tertiary care centre in Montreal, Canada. All cases met the Clark diagnostic criteria, which comprise 1) sudden cardiorespiratory arrest or both hypotension and respiratory compromise, 2) disseminated intravascular coagulation, 3) clinical onset during labour or within 30 min of delivery of the placenta, and 4) absence of fever. Two patients had a cardiac arrest and the third developed significant hypotension and hypoxia. In all patients, point-of-care echocardiography at the time of shock revealed signs of right ventricular failure including a D-shaped septum, acute pulmonary hypertension, and right ventricular systolic dysfunction. CONCLUSION: This case series and literature review of AFE emphasizes the importance of echocardiography in elucidating the etiology of maternal shock. The presence of right ventricular failure may be considered an important criterion to diagnose AFE.

RéSUMé: OBJECTIF: L'embolie de liquide amniotique (ELA) est une complication rare mais souvent fatale caractérisée par une instabilité hémodynamique et une coagulopathie soudaines survenant pendant le travail obstétrical ou au début de la période postpartum. Étant donné que les mécanismes menant au choc et les effets cardiovasculaires de l'ELA ne sont que partiellement compris, le but de cette série de cas était de décrire comment l'ELA apparaît à l'échocardiographie et de passer en revue les rares comptes rendus dans la littérature. CARACTéRISTIQUES CLINIQUES: Nous décrivons trois cas d'ELA survenus à l'Hôpital général juif, un centre tertiaire de soins à Montréal, au Canada. Tous les cas remplissaient les critères diagnostiques de Clark, qui comportent 1) un arrêt cardiorespiratoire soudain ou une hypotension accompagnée d'une détresse respiratoire, 2) une coagulation intravasculaire disséminée, 3) une apparition clinique pendant le travail obstétrical ou dans un délai de 30 minutes suivant la délivrance du placenta, et 4) l'absence de fièvre. Deux patientes ont subi un arrêt cardiaque et le tiers des patientes ont manifesté une hypotension et une hypoxie significatives. Chez toutes les patientes, l'échocardiographie au chevet au moment du choc a révélé des signes d'insuffisance ventriculaire droite, y compris un septum en forme de D, une hypertension pulmonaire aiguë et une dysfonction systolique ventriculaire droite. CONCLUSION: Cette série de cas et revue de littérature de l'ELA souligne l'importance de l'échocardiographie pour élucider l'étiologie du choc maternel. La présence d'une insuffisance ventriculaire droite peut être considérée un critère important pour diagnostiquer une ELA.

Dyssegmental dysplasia, Silverman-Handmaker type: A challenging antenatal diagnosis in a dizygotic twin pregnancy.

BACKGROUND: Dyssegmental dysplasia Silverman-Handmaker (DDSH; MIM 224410) type is an extremely rare skeletal dysplasia caused by functional null mutations in the perlecan gene. Less than forty cases are reported in the literature, of which only four were prenatally detected. METHODS: We report on a dizygotic twin pregnancy from consanguineous parents for which one of the twins presented prenatally with severe micromelia, limb bowing and scoliosis, and postnatally with clinical and radiological features compatible with a diagnosis of dyssegmental dysplasia. Molecular studies were undertaken to confirm the clinical diagnosis of DDSH. RESULTS: Molecular analysis results revealed a novel homozygous variant in the HSPG2 gene (MIM 142461), NM_005529.6(HSPG2):c.4029 + 1G>A, consistent with a diagnosis of DDSH. CONCLUSION: To the best of our knowledge, the current report is only the seventh molecularly confirmed case of DDSH.

Pregnancy as a possible trigger for heritable pulmonary arterial hypertension.

It is unclear whether pregnancy is a trigger or accelerant for idiopathic pulmonary arterial hypertension (PAH). Alternatively, its frequency close to the onset of symptoms and diagnosis in the idiopathic PAH population may represent a coincidence in a disease that predominates in young women. We describe a carrier of a BMPR2 gene mutation who had an uneventful first pregnancy but had aggressive PAH during her second pregnancy and now has ongoing heritable PAH. The possible role of pregnancy as a trigger in this vulnerable patient is discussed. Databases of patients with heritable PAH should be explored to see whether pregnancy is related to overt manifestation of the disease.

Cohort profile: the maternal-infant research on environmental chemicals research platform.

BACKGROUND: The Maternal-Infant Research on Environmental Chemicals (MIREC) Study was established to obtain Canadian biomonitoring data for pregnant women and their infants, and to examine potential adverse health effects of prenatal exposure to priority environmental chemicals on pregnancy and infant health. METHODS: Women were recruited during the first trimester from 10 sites across Canada and were followed through delivery. Questionnaires were administered during pregnancy and post-delivery to collect information on demographics, occupation, life style, medical history, environmental exposures and diet. Information on the pregnancy and the infant was abstracted from medical charts. Maternal blood, urine, hair and breast milk, as well as cord blood and infant meconium, were collected and analysed for an extensive list of environmental biomarkers and nutrients. Additional biospecimens were stored in the study's Biobank. The MIREC Research Platform encompasses the main cohort study, the Biobank and follow-up studies. RESULTS: Of the 8716 women approached at early prenatal clinics, 5108 were eligible and 2001 agreed to participate (39%). MIREC participants tended to smoke less (5.9% vs. 10.5%), be older (mean 32.2 vs. 29.4 years) and have a higher education (62.3% vs. 35.1% with a university degree) than women giving birth in Canada. CONCLUSIONS: The MIREC Study, while smaller in number of participants than several of the international cohort studies, has one of the most comprehensive datasets on prenatal exposure to multiple environmental chemicals. The biomonitoring data and biological specimen bank will make this research platform a significant resource for examining potential adverse health effects of prenatal exposure to environmental chemicals.

Maternal glucose tolerance in pregnancy affects fetal insulin sensitivity.

OBJECTIVE: Offspring of mothers with impaired glucose tolerance are far more likely to develop type 2 diabetes. We tested the hypothesis that maternal glucose tolerance in pregnancy affects fetal insulin sensitivity or beta-cell function. RESEARCH DESIGN AND METHODS: In a prospective singleton pregnancy cohort study, we analyzed glucose, insulin, and proinsulin concentrations in maternal blood at the 50-g oral glucose tolerance test (OGTT) at 24-28 weeks of gestation and in venous cord blood (n = 248). The cord blood glucose-to-insulin ratio and proinsulin concentration were used as indicators of fetal insulin sensitivity and the proinsulin-to-insulin ratio was used as an indicator of fetal beta-cell function. RESULTS: Higher OGTT blood glucose levels were associated with significantly lower cord plasma glucose-to-insulin ratios (r = -0.31, P < 0.001) and higher proinsulin concentrations (r = 0.31, P < 0.001) but not with proinsulin-to-insulin ratios. In a comparison of gestational diabetic (n = 26) versus euglycemic pregnancy, cord blood glucose-to-insulin ratios were substantially lower (geometric mean 10.1 vs. 20.0 mg/dl/microU/ml; P < 0.001), whereas proinsulin concentrations were much higher (24.4 vs. 13.8 pmol/l; P < 0.001), despite similar cord blood glucose concentrations indicating adequate management of diabetes. The differences remained significant after controlling for prepregnancy and fetal adiposity, family history of diabetes, gestational age, and other potential confounders. Significant changes in the glucose-to-insulin ratio and proinsulin concentration were also observed in obese (n = 31) mothers, but the differences became not statistically significant after adjustment for maternal glucose tolerance and fetal adiposity. CONCLUSIONS: Maternal glucose intolerance may impair fetal insulin sensitivity (but not beta-cell function) and consequently "program" the susceptibility to type 2 diabetes.

An international trial of antioxidants in the prevention of preeclampsia (INTAPP).

OBJECTIVE: We sought to investigate whether prenatal vitamin C and E supplementation reduces the incidence of gestational hypertension (GH) and its adverse conditions among high- and low-risk women. STUDY DESIGN: In a multicenter randomized controlled trial, women were stratified by the risk status and assigned to daily treatment (1 g vitamin C and 400 IU vitamin E) or placebo. The primary outcome was GH and its adverse conditions. RESULTS: Of the 2647 women randomized, 2363 were included in the analysis. There was no difference in the risk of GH and its adverse conditions between groups (relative risk, 0.99; 95% confidence interval, 0.78-1.26). However, vitamins C and E increased the risk of fetal loss or perinatal death (nonprespecified) as well as preterm prelabor rupture of membranes. CONCLUSION: Vitamin C and E supplementation did not reduce the rate of preeclampsia or GH, but increased the risk of fetal loss or perinatal death and preterm prelabor rupture of membranes.

Should we offer expectant management in cases of severe preterm preeclampsia with fetal growth restriction?

OBJECTIVE: The purpose of this study was to assess maternal and fetal morbidity and death in cases of severe preterm preeclampsia that were managed expectantly. STUDY DESIGN: It is a retrospective study that included 155 singleton pregnancies with severe preeclampsia at <34 weeks of gestation that were managed expectantly over a 10-year period. Perinatal outcomes of both mother and fetus were stratified according to gestational age and the severity of fetal growth restriction < or =3th percentile, 4th to 5th percentile, >5th to10th percentile, and >10th percentile. RESULTS: The mean gestational age at admission was 30.2 +/- 2.4 weeks (range, 23.9-34.0 weeks). The mean latency period was 5.3 +/- 5.2 days, with a perinatal mortality rate of 3.9%. Gestational age of <30 weeks of gestation was the strongest variable that affected perinatal outcome, whereas fetal growth restriction played a marginal role. CONCLUSION: Expectant management is recommended strongly in fetuses at <30 weeks of gestation, irrespective of fetal growth restriction. Delivery should be considered at >30 weeks of gestation.