Mode of delivery and peripartum outcome in women with heart disease according to the ESC guidelines: an Italian multicenter study.

INTRODUCTION: The European Society of Cardiology (ESC) guidelines (GL) provide indications on the mode of delivery in women with heart disease. However available data suggests that the rate of Cesarean Delivery (CD) is high and widely variable among such patients. In this study, we aimed to investigate the degree of adherence to the ESC recommendations among women delivering in four tertiary maternity services in Italy and how this affects the maternal and neonatal outcomes. MATERIAL AND METHODS: Retrospective multicenter cohort study including pregnant women with heart disease who gave birth between January 2014 and July 2020. Composite adverse maternal outcome (CAM) was defined by the occurrence of one or more of the following: major postpartum hemorrhage, thrombo-embolic or ischemic event, de novo arrhythmia, heart failure, endocarditis, aortic dissection, need for re-surgery, sepsis, maternal death. Composite Adverse Neonatal outcome (CAN) was defined as cord arterial pH <7.00, APGAR <7 at 5 min, admission to the intensive care unit, and neonatal death. We compared the incidence of CAM and CAN between the cases with planned delivery in accordance (group "ESC consistent") or in disagreement (group "ESC not consistent") with the ESC GL. RESULTS: Overall, 175 women and 181 liveborn were included. A higher frequency of CAN was found when delivery was not planned accordingly to the ESC guidelines [("ESC consistent" 9/124 (7.2%) vs "ESC not consistent" 13/57 (22.8%) p = 0.002 OR 3.74 (CI 95% 1.49-9.74) , while the occurrence of CAM was comparable between the two groups. At logistic regression analysis, the gestational age at delivery was the only parameter independently associated with the occurrence of CAN (p = 0.006). CONCLUSION: Among pregnant women with heart disease, deviating from the ESC guidelines scheduling cesarean delivery does not seem to improve maternal outcomes and it is associated with worse perinatal outcomes, mainly due to lower gestational age at birth.

Prenatal visualisation of the torcular herophili by means of a Doppler technology highly sensitive for low-velocity flow in the expert assessment of the posterior fossa: a prospective study.

OBJECTIVE: To evaluate the usefulness of a Doppler technology highly sensitive for low-velocity flow in the antenatal imaging of the torcular herophili (TH) in the second trimester of pregnancy. DESIGN: Prospective study. SETTING: Referral Fetal Medicine Unit. POPULATION: Non-consecutive series of singleton pregnancies submitted to antenatal neurosonogram between 20 and 28 weeks of gestation. METHODS: A midsagittal section of the fetal brain was obtained by insonating through the anterior fontanelle, then the MV-Flow™ and LumiFlow™ presets were selected to visualise the TH as the posterior confluence of the superior sagittal sinus and the straight sinus. MAIN OUTCOME MEASURES: Evaluation of the anatomic relationship of the TH with the 'transpalatal line' joining the upper bony palate to the fetal skull. RESULTS: A total of 99 pregnant women were recruited, including one fetus with open spina bifida, one with Dandy-Walker malformation (DWM) and two with Blake's pouch cysts. In normal fetuses, the TH appeared to lie on or just below the 'transpalatal line'. In the cases of Blake's pouch cyst, the position of the TH appeared normal if compared with controls, whereas in DWM a supra-elevated position of the TH in respect of the transpalatal line was demonstrated. Finally, in the fetus with Chiari II malformation the TH was identified below the 'transpalatal plane'. CONCLUSIONS: Prenatal ultrasound visualisation of the TH by means of newly developed Doppler technologies characterised by high sensitivity for low-velocity flow is feasible and allows the indirect evaluation of the insertion of cerebellar tentorium in the second trimester. TWEETABLE ABSTRACT: Prenatal imaging of the torcular herophili using a Doppler technology highly sensitive for low-velocity flow.

Intracervical lakes as sonographic marker of placenta accreta spectrum disorder in patients with placenta previa or low-lying placenta.

OBJECTIVE: To evaluate the diagnostic accuracy of a new ultrasound sign, intracervical lakes (ICL), in predicting the presence of placenta accreta spectrum (PAS) disorder and delivery outcome in patients with placenta previa or low-lying placenta. METHODS: This was a retrospective multicenter study of women with placenta previa or low-lying placenta at ≥ 26 weeks' gestation, who were referred to three Italian tertiary units from January 2015 to September 2018. The presence of ICL, defined as tortuous anechoic spaces within the cervix which appeared to be hypervascular on color Doppler, was evaluated on ultrasound images obtained at the time of referral. The primary aim was to explore the diagnostic accuracy of ICL in detecting the presence and depth of PAS disorder. The secondary aim was to explore the accuracy of this sign in predicting total estimated blood loss, antepartum bleeding, major postpartum hemorrhage at the time of Cesarean section and need for Cesarean hysterectomy. The diagnostic accuracy of ICL in combination with typical sonographic signs of PAS disorder, was assessed by computing summary estimates of sensitivity, specificity, positive and negative predictive values, positive and negative likelihood ratios and diagnostic odds ratios (DOR). RESULTS: A total of 332 women with placenta previa or low-lying placenta were included in the analysis, with a median maternal age of 33.0 (interquartile range, 29.0-37.0) years. ICL were noted in 15.1% of patients. On logistic regression analysis, the presence of ICL was associated independently with major postpartum hemorrhage (odds ratio (OR), 3.3 (95% CI, 1.6-6.5); P < 0.001), Cesarean hysterectomy (OR, 7.0 (95% CI, 2.1-23.9); P < 0.001) and placenta percreta (OR, 2.8 (95% CI, 1.3-5.8); P ≤ 0.01), but not with the presence of any PAS disorder (OR, 1.6 (95% CI, 0.7-3.5); P = 0.2). Compared with the group of patients without ultrasound signs of PAS disorder, the presence of at least one typical sonographic sign of PAS disorder in combination with ICL had a DOR of 217.2 (95% CI, 27.7-1703.4; P < 0.001) for placenta percreta and of 687.4 (95% CI, 121.4-3893.0; P < 0.001) for Cesarean hysterectomy. CONCLUSION: ICL may represent a marker of deep villus invasion in women with suspected PAS disorder on antenatal sonography and anticipate the occurrence of severe maternal morbidity. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Los espacios intracervicales como marcador ecográfico de trastornos del espectro de la placenta acreta en pacientes con placenta previa o placenta baja OBJETIVO: Evaluar la precisión del diagnóstico de un nuevo indicio de ultrasonido, los espacios intracervicales (EIC), para predecir la presencia de trastornos del espectro de la placenta acreta (EPA) y el resultado del parto en pacientes con placenta previa o placenta baja. MÉTODOS: Este fue un estudio multicéntrico retrospectivo de mujeres con placenta previa o placenta baja a ≥ 26 semanas de gestación, que se remitieron a tres unidades terciarias italianas desde enero de 2015 a septiembre de 2018. Se evaluó la presencia de EIC, definida como espacios anecoicos tortuosos dentro del cuello uterino que parecían ser hipervasculares en el Doppler a color, en imágenes de ecografías obtenidas en el momento de la remisión. El objetivo principal fue estudiar la precisión del diagnóstico mediante EIC en la detección de la presencia y la profundidad de un trastorno del EPA. El objetivo secundario fue explorar la precisión de este indicador para predecir la pérdida total estimada de sangre, la hemorragia antes del parto, la hemorragia puerperal importante en el momento de la cesárea y la necesidad de una histerectomía por cesárea. La precisión diagnóstica de EIC, en combinación con los indicios ecográficos típicos de los trastornos del EPA, se evaluó calculando estimaciones estadísticas descriptivas de la sensibilidad, la especificidad, los valores predictivos positivos y negativos, los cocientes de verosimilitud positivos y negativos y las razones de momios del diagnóstico (RMD). RESULTADOS: En el análisis se incluyó un total de 332 mujeres con placenta previa o placenta baja, con una mediana de la edad materna de 33,0 años (rango intercuartil, 29,0-37,0). Se observaron EIC en el 15,1% de las pacientes. En el análisis de regresión logística, la presencia de EIC se asoció de forma independiente con la hemorragia puerperal grave (razón de momios (RM), 3,3 (IC 95%, 1,6-6,5); P<0,001), la histerectomía por cesárea (RM, 7,0 (IC 95%, 2,1-23,9); P<0,001) y la placenta percreta (RM, 2,8 (IC 95%, 1,3-5,8); P≤0,01), pero no con la presencia de ningún trastorno del EPA (RM, 1,6 (IC 95%, 0,7-3,5); P=0,2). En comparación con el grupo de pacientes sin indicios de ultrasonido de algún trastorno del EPA, la presencia de al menos un indicio ecográfico típico de trastorno del EPA en combinación con EIC tuvo una RMD de 217,2 (IC 95%, 27,7-1703,4; P<0,001) para la placenta percreta y de 687,4 (IC 95%, 121,4-3893,0; P<0,001) para la histerectomía por cesárea. CONCLUSIÓN: Los EIC pueden representar un marcador de invasión profunda de las vellosidades en mujeres con sospecha de algún trastorno del EPA basado en la ecografía prenatal y anticipar la presencia de una morbilidad materna grave.

Perinatal morbidity and mortality in early-onset fetal growth restriction: cohort outcomes of the trial of randomized umbilical and fetal flow in Europe (TRUFFLE).

OBJECTIVES: Few data exist for counseling and perinatal management of women after an antenatal diagnosis of early-onset fetal growth restriction. Yet, the consequences of preterm delivery and its attendant morbidity for both mother and baby are far reaching. The objective of this study was to describe perinatal morbidity and mortality following early-onset fetal growth restriction based on time of antenatal diagnosis and delivery. METHODS: We report cohort outcomes for a prospective multicenter randomized management study of fetal growth restriction (Trial of Randomized Umbilical and Fetal Flow in Europe (TRUFFLE)) performed in 20 European perinatal centers between 2005 and 2010. Women with a singleton fetus at 26-32 weeks of gestation, with abdominal circumference < 10(th) percentile and umbilical artery Doppler pulsatility index > 95(th) percentile, were recruited. The main outcome measure was a composite of fetal or neonatal death or severe morbidity: survival to discharge with severe brain injury, bronchopulmonary dysplasia, proven neonatal sepsis or necrotizing enterocolitis. RESULTS: Five-hundred and three of 542 eligible women formed the study group. Mean ± SD gestational age at diagnosis was 29 ± 1.6 weeks and mean ± SD estimated fetal weight was 881 ± 217 g; 12 (2.4%) babies died in utero. Gestational age at delivery was 30.7 ± 2.3 weeks, and birth weight was 1013 ± 321 g. Overall, 81% of deliveries were indicated by fetal condition and 97% were by Cesarean section. Of 491 liveborn babies, outcomes were available for 490 amongst whom there were 27 (5.5%) deaths and 118 (24%) babies suffered severe morbidity. These babies were smaller at birth (867 ± 251 g) and born earlier (29.6 ± 2.0 weeks). Death and severe morbidity were significantly related to gestational age, both at study entry and delivery and also with the presence of maternal hypertensive morbidity. The median time to delivery was 13 days for women without hypertension, 8 days for those with gestational hypertension, 4 days for pre-eclampsia and 3 days for HELLP syndrome. CONCLUSIONS: Fetal outcome in this study was better than expected from contemporary reports: perinatal death was uncommon (8%) and 70% survived without severe neonatal morbidity. The intervals to delivery, death and severe morbidity were related to the presence and severity of maternal hypertensive conditions.

Severe brain damage from twin-twin transfusion syndrome treated with serial amnioreductions after 26 weeks: a case to reconsider the gestational age limits of laser therapy.

We report a case of monochorionic twin pregnancy complicated by twin-twin transfusion syndrome (TTTS) diagnosed in the late second trimester and treated with two amnioreductions. Three days after the first amniodrainage, the recipient twin developed intracranial ventriculomegaly and, similarly, after a few days, the donor showed signs of brain damage at MRI. We discuss the possible mechanism of brain damage of amnioreductions performed after 26 weeks of gestation in a monochorionic pregnancy with TTTS as a result of a placental 'steal' phenomenon.

Mid- and long-term outcome of extremely low birth weight (ELBW) infants: an analysis of prognostic factors.

OBJECTIVE: To evaluate morbidity and long-term neurological outcome in a group of extremely low birth weight infants (ELBW; <1000 g) and to correlate the neurological outcome in a small group of intrauterine growth retarded (IUGR) infants with Doppler indices in the umbilical artery. METHODS: One hundred and eighty-three live births with birth weight <1000 g and gestational age < or=34 weeks were included in the study. Neonatal mortality and morbidity were evaluated. At 24 months of corrected age an evaluation of the neurological development of the children was made by pediatric neuropsychiatrists. The children were classified as: normal, with minor neurological sequelae, and with major neurological sequelae. The evaluation of umbilical artery velocimetry was applied to 84 fetuses presenting with IUGR and the velocimetric patterns were correlated with neurological outcome. RESULTS: In the 183 infants discharged from the Department of Neonatology, respiratory distress syndrome (RDS) was the most frequent pathology (76.6%); less frequent were bronchopulmonary dysplasia (BPD; 19.5%), patent ductus arteriosus (PDA; 29.7%) and necrotizing enterocolitis (NEC; 5.5%). Retinopathy of prematurity (ROP) affected 34 children (26.6%), and 14.8% of the children developed intraventricular hemorrhage (IVH) and 14.1% periventricular leukomalacia (PVL). Out of the 183 infants included in the study, 107 had a neurological assessment at two years: 22 (20.6%) suffered from severe neurological sequelae, 20 (18.7%) from minor neurological sequelae, and 65 (60.7%) had a normal neurological development. In 84 IUGR fetuses a Doppler evaluation of the umbilical artery was performed: the incidence of neurologically normal children was 67% in the group with normal umbilical velocimetry, 93% in the group with increased umbilical resistances, and 59% in those with absent or reversed end-diastolic velocity (ARED). CONCLUSIONS: This study, confirms that an extremely low birth weight implies a high risk of perinatal mortality and neonatal morbidity, but that the most significant variable that can be correlated to the long-term neurological outcome is the gestational age.

In-utero transplantation of parental CD34 haematopoietic progenitor cells in a patient with X-linked severe combined immunodeficiency (SCIDXI).

BACKGROUND: X-linked severe combined immunodeficiency (SCIDXI) is an inherited immune defect which leads to death in infancy from severe infections. The defect is caused by mutations of the IL-2RG gene that encodes for the common gamma chain shared by several cytokine receptors. The disease is characterised by lack of T and NK cells with normal numbers of B cells. SCIDXI can be cured by bone marrow transplantation (BMT) or prevented by abortion after prenatal diagnosis. METHODS: A male fetus was diagnosed as having SCIDXI by molecular, immunophenotypic, and functional analyses. The fetus was injected intraperitoneally under ultrasound guidance with CD34 haematopoietic progenitor cells purified from paternal bone marrow and T-cell depleted by E rosetting. Chimerism analysis was by HLA-DQ alpha typing and gamma-chain staining on cord blood. FINDINGS: A healthy 3.6 kg boy was delivered by caesarean section at 38 weeks of gestation with no clinical or laboratory signs of graft-versus-host disease. Engraftment of donor-derived CD2 cells was found at birth. At 3.5 months of age the infant is well and his T-cell counts and function are normal. INTERPRETATION: In-utero transplantation of haematopoietic progenitor cells allowed immune reconstitution of a fetus with SCIDXI and may be an alternative to elective abortion. Our report should encourage applications of this method to other inherited disorders curable by BMT.