E. Gómez Montes

EP12.31: Prenatal prognosis markers in congenital diaphragmatic hernia

Objectives: To assess the ability of prenatal prognostic markers to predict the postnatal outcome in fetuses diagnosed with congenital diaphragmatic hernia (CDH). Methods: Retrospective study of fetuses diagnosed with CDH between 2006-2016 at a tertiary care referral centre. Following prenatal prognostic markers were analysed: lung-to-head ratio (LHR), observed-to-expected LHR (O/E-LHR), right CDH, liver herniation (LH), associated congenital anomalies/chromosomopathies, and gestational age (GA) at diagnosis. We compared these parameters with the postnatal outcome [pulmonary hypertension (PH), need for extracorporeal membrane oxygenation (ECMO), survival at discharge and survival at 6 months of life]. Results: Diagnosis of CDH was established in 54 cases of CDH. Twenty-six cases were excluded because of termination of pregnancy (TOP). One of them, at the time of the study, the newborn was still admitted to the Pediatric Intensive Care Unit, with one month of life. Of the remaining 27 cases and according to the postnatal outcome, 52.6% (10/19) developed PH, 87.5% (21/24) needed ECMO, 55.6% (15/27) survived at hospital discharge and 48.1% (13/27) survived at 6 months of life. No differences in the prognostic markers were found regarding to PH and need for ECMO after birth. Those patients who did not survive at discharge and at 6 months of life had a significantly higher rate of LHR <1 (86% vs. 14%, p=0.033 and 100% vs. 0%, p=0.007, respectively) and LH (69% vs. 31%, p=0.017 and 77% vs. 23%, p=0.016). No survivors at 6 months of life were also significantly earlier diagnosed (23.6±6 vs. 28.3±6, p=0.048) and had more frequently associated anomalies (80% vs. 20%, p=0.021). We did not find any differences for the remaining prognostic markers regarding survival at discharge neither at 6 months of life. Conclusions: Prognostic markers associated with a poorer postnatal outcome were early GA at diagnosis, LH, LHR<1 and associated anomalies. These data are according to reported.

P21.02: Interrupted aortic arch in foetal live

knowing the strengths and weaknesses of alternate biometric standards. Varied choices of fetal biometry charts 1. disrupt continuity of care for pregnant women 2. magnify the likelihood of false positive abnormal fetal biometry 3. produce ‘‘noise’’ which can obscure true signal of abnormal fetal biometry thus obscuring conditions such as fetal growth restriction, macrosomia, microcephaly and skeletal dysplasia. Practitioners recognise these hazards but require guidance to minimise them. As no ideal Australasian fetal biometry chart have been published, new, regionally appropriate reference range should be developed from a representative sample, using sound statistical techniques, especially to model the clinically important extremes of reference ranges i.e. 3rd centile and standard deviation for head circumference, abdominal circumference and femur length.

OC02.05: Fetal cardiac intervention: 8-year experience

Objectives: To assess blood flow measurements of the fetal aorta performing cardiac MRI using a newly developed MR compatible Doppler-ultrasound device for triggering of the fetal heart in utero and to compare them with ultrasound studies in a sheep model. Methods: Four pregnant sheep carrying singleton fetuses (123 days gestational age) underwent ultrasound examination for determination of blood flow velocity in the fetal aorta descendens. The ewes were then anesthetized to undergo fetal MRI examination on a 1.5 T imager. A newly developed MR-compatible Doppler-ultrasound sensor was placed on the abdomen of the ewe, above the fetal heart and fixed with a belt. The recorded signal of the fetal heart was transferred to the ECG trigger unit of the MR scanner and used for cardiac triggering. Blood flow velocity measurements of the fetal aorta descendens were performed and compared to ultrasound measurements. Mean values and peak velocities were calculated. Results: Triggering of the fetal heart rate was possible in all examinations. Using the trigger signal excellent MR images of the fetal heart and high quality flow measurements of the fetal aorta were obtained. Comparison of both methods revealed no significant differences with mean peak flow velocities of 60 cm/s (± 3.4) and 62 cm/s (± 9.2). Conclusions: Blood flow measurements of the fetal aorta were successfully performed using the newly developed MR-compatible Doppler-ultrasound device for fetal cardiac triggering in a sheep model, revealing no significant differences compared to fetal ultrasound examination. The newly developed Doppler-ultrasound device for triggering fetal cardiovascular MRI enables precise intrauterine fetal blood flow measurements and might be an alternative to fetal ultrasound.

P25.02: Isolated aberrant right subclavian artery: a marker of trisomy 21

A persistent cloaca, a rare congenital anomaly, is single channel made by confluence of rectum, vagina, urinary tract. Sonographic finding of cloaca including urinary tract malformation with secondary change such as hydronephrosis, hydroureter or dysplastic kidneys, dilated bowel, cystic pelvic mass is very difficult. We report a case of concomitant anorectal, urogenital, and mullerian anomalies mimicking persistent cloaca. An 35-year-old G2P1 women was referred to our institute at 33weeks of gestation presenting of fetal ascites, hydronephrosis. On ultrasound 7.8 × 5.3 cm sized septated cystic mass occupying whole abdomen and pelvis was noted. Abdominal wall was intact and ascites was not noted. Bilateral hydronephrosis(grade III) was found but bladder was not visible. In addition, absence of anal rim and ambiguous genitalia was suspected. Based on ultrasonographic finding, cloacal malformation was suspected. At 36 weeks of gestation, repeat cesarean section was performed due to oligohydramnios to deliver a female infant weight 2655gm with Apgar score of 5 and 6 at 1 and 5 minutes, respectively. Postnatal diagnosis based on evaluations including sonography and voiding cystourethrogram, antegrade pyelonephrography, computed tomography, surgical exploration was rectovesical fistula, vaginal outlet obstruction, ectopic ureteral insertion to vaginal wall, imperforated anus. Huge cystic mass thought to be persistent cloaca was severe hydrometrocolpos resulting from vaginal outlet obstruction and ureterovaginal fistula.

OP04.03: Prediction of coarctation of the aorta in the second half of pregnancy

6–10 10 1 1 4 2 7 2 3 2 1 1 1 8 2 6 51 10–12 8 2 5 11 8 16 1 12 7 9 6 1 2 1 3 4 4 6 106 12–14 7 1 10 18 1 10 24 1 11 4 1 5 6 5 10 3 1 1 1 1 3 124 14–16 3 1 13 8 3 10 18 13 7 5 5 2 8 4 7 2 1 3 113 16–20 11 9 14 10 4 3 6 57 20–24 10 4 12 7 2 3 3 41 24–28 3 2 7 3 1 16 28–32 2 2 6 2 1 3 16 32–40 3 3 1 2 3 12 Total/Class 28 5 28 29 38 8 30 15 65 2 38 28 21 3 19 33 18 9 20 12 8 4 13 9 15 4 18 16 Total System 90 91 133 76 59 34 53 536

OC19.03: Prediction of postnatal outcome of tetralogy of Fallot at mid‐second trimester of pregnancy

corrected age in infants who had been complicated by growthrestricted in utero and very low birth weight (VLBW). Methods: Sixty-four singleton pregnancies complicated by both fetal growth restriction (FGR) and estimated fetal weight (EFW) below 1500 g were observed. Multiple pregnancies and fetal anomalies were excluded. Still birth and neonatal-infantile death were excluded. FGR was defined as EFW below −1.5 SD. EFW and SD were calculated by local standards (JSUM). The relationships of umbilical artery (UA), middle cerebral artery (MCA), gestational age at delivery (GA), EFW and SD with a 3-year neurodevelopmental delay were evaluated. In 64 infants at corrected 3 years, the Kyoto Scale of Psychological Development (KSPD) was administered by trained psychologists. KSPD is standard developmental test for Japanese children. KSPD was assessed by developmental quotient (DQ) in the following three areas: postural-motor (PM), cognitiveadaptive (CA) and language-social (LS). A total DQ was obtained by PM-DQ, CA-DQ and LS-DQ. Developmental outcome was classed as delay for DQ for KSPD < 70. Results: There was no case complicated with blindness and/or hearing loss. In total DQ, neurodevelopmental delay was associated with EFW below 600 g (P = 0.048, AUC:0.674) and GA below 27 weeks (P = 0.027, AUC:0.694). PM delay was associated with EFW below 700 g and GA below 27 weeks. CM delay was associated with GA below 27 weeks. LS delay was associated with EFW below 600 g and GA below 27 weeks. In all categories, Z scores, UA Doppler and MCA Doppler was not associated with neurodevelopmental delay. Conclusions: GA and EFW remain the predominant factors for poor neurodevelopment in FGR and VLBW fetuses.

OP09.03: First and second trimester uterine artery Doppler study in high-risk women for preeclampsia

Objectives: To evaluate the utility of sequential measurement of the uterine artery (UtA) resistances in both the 11–14 weeks scan (1T) and the 19–22 weeks scan (2T) for the prediction of preeclampsia (PE) in a high-risk population. Methods: Prospective observational study of 155 singleton pregnancies at high risk for PE (at least having one major risk factor: prior PE, chronic hypertension, pregestational diabetes mellitus, chronic renal disease, BMI > 30, autoimmune disorders or thrombophilia). Pregnancies with abnormal karyotype or incomplete outcome were excluded. Measurement of mean UtA pulsatility index (UtA-PI) was performed at 1T and 2T and statistical comparisons were established between unaffected gestations and those who developed either late PE (gestational age at delivery ≥ 34 weeks) or early PE (< 34 weeks). Results: Late PE developed in 21 (13.5%) pregnancies and early PE in six (3.9%). No severe complications were noticed in the late PE group, while two cases of abruption and one case of intrauterine death occurred in the early PE group. 90th centile of the mean UtA-PI in the 1T and 2T were 2.25 and 1.50, respectively. Using these cut-off values, the detection rates of late and early PE in the 1T were 14.3% and 17.0%, respectively, and in the 2T were 19.0% and 66.7%, respectively. Noteworthy, 6/9 (67%) pregnancies with mean UtA-PI > 1.8 in the 2T developed PE (2 late, 4 early) (OR 13.9; 95% CI 3.2–59.8), and 5/14 (36%) pregnancies with mean UtA-PI in the 2T > mean UtA-PI in the 1T developed PE (2 late, 3 early) (OR 8.2; 95% CI 2.1–33.0). Conclusions: When applied to high-risk women, UtA Doppler screening proved to be ineffective for the prediction of late PE and showed a modest performance for the prediction of early PE, especially in the 1T. However, very high UtA resistances in the 2T as well as the increase of the UtA resistances between 1T and 2T are associated with a highly increased risk of developing PE in high-risk women. OP09.04 Maternal ophthalmic artery Doppler: novel parameter to predict early-onset pre-eclampsia in the first trimester of pregnancy

OP35.10: Pulmonary atresia/critical stenosis with intact ventricular septum: prediction of outcome in the second trimester of pregnancy

Objectives: To analyze the methods and echocardiographic characteristics of all kinds of aortic arch anomalies and to improve prenatal diagnostic accuracy. Methods: From 2004 to 2007, third-trimester screening of 41900 fetuses were performed in our hospital. 486 fetuses with congenital heart anomalies were diagnosed including 73 fetuses with congenital aortic arch anomalies. 73 fetuses with aortic arch abnormalities were classified by three aspects: aortic arch position anomalies relative to the trachea, the number of aortic arch anomalies, abnormal aortic arch diameter anomalies and aortic arch branch anomalies. We use different view to diagnosis aortic arch anomalies, including three vascular trachea view (3VT), trachea coronal view, thoracic aorta coronal view, longitudinal view of the aortic arch and longitudinal view of the ductus arch. Results: The details of 73 cases with congenital aortic arch anomalies were: 32 fetuses with malposition of aortic arch, 1 fetus with double aortic arch, 9 fetuses with normal left aortic arch and aberrant left subclavian artery, 5 fetuses with interrupted aortic arch, 26 fetuses with coarctation of aortic arch. 16 cases of 73 cases were done a chromosome examination, 7 cases which found chromosome abnormal were all 18-trisomy syndrome. The accuracy rate of prenatal sonographic diagnosis of congenital aortic arch anomalies was 86.36%. Conclusions: All kinds of aortic arch anomalies have typical sonographic image. If we know characteristics of all kinds of aortic arch anomalies, we could improve prenatal diagnostic accuracy. It is very useful to find and diagnosis aortic anomalies by using 3VT and trachea coronal view. It is also important to diagnosis interrupted aortic arch and coarctation of aortic arch by using color Doppler flow imaging. But coarctation of aortic arch was difficult diagnosed by prenatal ultrasound examination. Because it was very difficult to distinguish from interrupted aortic arch.

OP32.05: Prenatal diagnosis of neonatal Marfan syndrome

Marfan syndrome (MS) is a rare disease of the connective tissue. Neonatal MS is even rarer, and rarer still is its prenatal ultrasound diagnosis with 7 cases reported and only 1 surviving patient. We describe 2 new cases. Both had no relevant family history, had normal scans in the first half of pregnancy, were referred because of fetal cardiomegaly at 28 and 35 weeks respectively, and were delivered at term. Case 1: Fetal echocardiography showed severe cardiomegaly, dilation of the initial portion of the great vessels and mild prolapse of the atrioventricular (AV) valve leaflets. Postnatally, a marfanoid habitus was found and a moderate insufficiency of the AV and semilunar valves was also seen. The neonate was discharged on the 7th day and an angiotensin II receptor antagonist (Losartan) was prescribed to prevent aortic root dilation. Echocardiography at 2 months revealed severe AV valve insufficiency. Rapid worsening required AV valve surgical repair, but the patient died 5 days later. A heterozygous mutation of the fibrillin-1 (FBN1) gene was found. Case 2: Fetal echocardiography showed dilation of the outflow tracts, AV valve insufficiency and redundant AV cusps protruding in the atria during systole. Enlarged and hiperflexed hands and feet were observed. A scan 2 weeks later revealed aortic insufficiency. Postnatally, the same marfanoid habitus was noted. The patient was discharged at 31 days, with Losartan. Two weeks later the patient was readmitted, chest X-ray revealed severe cardiomegaly and emphysematous lungs, and death occurred soon after due to cardiorespiratory arrest. A new mutation of the FBN1 gene was found. The combination of cardiomegaly, enlargement of great vessels and multivalvular regurgitation in the second half of pregnancy may lead to suspect neonatal MF. These valve problems progress relentlessly, leading to heart failure and early death in most cases. This information may be helpful to provide the parents the most accurate counseling.