D. Escribano

Prenatal Detection of Congenital Heart Defects: A Survey on Clinical Practice in Spain

Introduction: Second-trimester fetal screening for congenital heart defects (CHD) included in routine obstetric care provides relevant information for decision making. The aim of this study was to describe the clinical practice of prenatal detection of CHD in terms of the process and results. Methods: The characteristics and results of ultrasound screening for major CHD were documented using data provided by hospitals for a national survey in Spain over the period of 2004–2006. Sixty-seven percent of eligible centers (56/83), covering 36% of total births nationwide, responded to the survey; 33 of these returned complete data regarding the screening results. Results: The number of major CHD occurring in the centers which provided data with results of screening was 1,060. The overall prenatal detection rate of major CHD was 65.7% (95% CI 57.8–74.7), but the detection rate in the routine second-trimester scan was 52.6% (95% CI 45.6–60.8). In 61% of these cases the parents chose to terminate the pregnancy. Two independent predictors of increased detection by center were identified: first, the uniformity and systematic character of the examination of the heart showing at least the 4-chamber view and outflow tracts (prevalence ratio 1.3, 95% CI 1.0–1.8) and second, the local availability of specialists in fetal echocardiography (prevalence ratio 1.4, 95% CI 1.1–1.9). Conclusions: The detection of major CHD in the first half of pregnancy has an important impact on parental decision making. The prenatal screening program for CHD should be globally strengthened in terms of qualifications and methodological approaches. To improve its performance locally, close collaboration with fetal heart specialists should be promoted.

Prediction of coarctation of the aorta in the second half of pregnancy

To determine which combination of cardiac parameters provides the best prediction of postnatal coarctation of the aorta (CoAo) in fetuses with cardiac asymmetry.

Uterine artery Doppler and sFlt‐1/PlGF ratio: prognostic value in early‐onset pre‐eclampsia

To evaluate the performance of the mean uterine artery pulsatility index (UtA‐PI) and the automated measurement of the soluble fms‐like tyrosine kinase‐1 (sFlt‐1)/placental growth factor (PlGF) ratio for the prognostic assessment of both maternal and perinatal outcomes, and the time‐to‐delivery interval in early‐onset (≤ 34 + 0 weeks) pre‐eclampsia (PE) cases with attempted expectant management.

Gestational age-specific scoring systems for the prediction of coarctation of the aorta

To determine which combination of cardiac parameters provides the best prenatal prediction of coarctation of aorta (CoAo).

Predictive value of sequential models of uterine artery Doppler in pregnancies at high risk for pre-eclampsia.

To evaluate the performance of models described previously for the prediction of pre‐eclampsia (PE), based on the sequential evaluation of uterine artery resistance at 11–13 weeks and 19–22 weeks, in a high‐risk population.

Transposition of the Great Arteries in Fetal Life: Accuracy of Diagnosis and Short-Term Outcome

Objective: To review our series of prenatally diagnosed transposition of the great arteries (TGA) to analyze the accuracy of fetal echocardiography for achieving a precise diagnosis of the TGA type (simple vs. complex) and to examine the short-term outcome. Methods: A total of 94 cases of simple and complex TGA types (ventriculoarterial discordance with atrioventricular concordance) prenatally evaluated in our referral center between 1998 and 2014 were included. Fetuses with additional congenital anomalies and those with incomplete follow-up were excluded. Prenatal diagnostic accuracy and short-term survival were analyzed for the different types of TGA. Results: The TGA type was correctly ascertained prenatally in 93.3%. Most fetuses were diagnosed with simple TGA (62.7%). There were 6 discrepancies: 5 fetuses with simple TGA had postnatally TGA + ventricular septal defect (VSD; n = 3) or TGA + VSD + coarctation of the aorta (n = 2), and 1 fetus with TGA + VSD postnatally showed severe left ventricular outflow tract obstruction. The mortality rate was 6.6%; it was higher in complex versus simple forms (12.8 vs. 1.9%, p = 0.038), and in cases with intramural coronary artery versus those without (60 vs. 3.5%, p < 0.001). We found no relationship between the arrangement of the great arteries and coronary arterial abnormalities. Conclusions: Simple TGA has a better outcome than the complex forms. A discrepancy rate of 7% with potential influence on the prognosis of survival between the prenatal diagnosis of the TGA type and the definitive diagnosis was found.

Impact of prenatal diagnosis of transposition of the great arteries on postnatal outcome

Abstract Objective: To assess the impact of prenatal diagnosis of transposition of the great arteries (TGA) on postnatal outcome. Methods: Hundred and fifty-four patients with either simple (n = 101) or complex forms (n = 53) of TGA, diagnosed prenatally (G1, n = 88) or postnatally (G2, n = 66), who were admitted and underwent surgical correction in our centre between 1998 and 2014, were analysed. Results: Prostaglandin E1 (PgE1) infusion and balloon atrial septostomy (BAS) were performed in the first 48 h after birth more commonly in G1. The hospital mortality rate for the whole group was 7.1%, higher for complex forms (13.2%) than for simple TGA (3.9%), (p = 0.034). The overall mortality rate was similar in G1 and G2. The mortality for simple TGA was higher when PgE1 infusion and BAS were implemented after the first 48 h (p = 0.001). All deaths in G2 occurred in patients first receiving PgE1 and BAS beyond 48 h. PgE1 was initiated in the first 48 h in most patients (83%) with simple TGA postnatally diagnosed. Conclusions: Adequate measures in the first 48 h after birth are essential to reduce the early mortality in TGA, especially in the simple form. This can be provided by prenatal diagnosis or by early neonatal clinical suspicion and prompt measures.

Fetal Aortic Valvuloplasty: Experience and Results of Two Tertiary Centers in Spain

Objective: Fetal aortic valvuloplasty (FAV) may avoid progression of critical aortic stenosis (CAS) to hypoplastic left ventricle, improving the options for biventricular circulation (BVC). We describe the results of FAV in 2 referral centers in Spain. Methods: We analyzed all FAVs performed in the period 2007-2015. The selection of candidates, the technique, and postnatal management were made following an agreed protocol. A descriptive analysis of survival, type of circulation after birth, and complications was made, considering all deaths in the first 48 h after FAV as FAV-related. Results: FAV was performed in 28 fetuses at a median gestational age (GA) of 23 weeks (range, 20-32). FAV was technically successful in 22 (78.6%), of whom 11 were born alive and with intention to treat. Eight (72.7%) resulted in BVC and 3 (27.3%) in univentricular circulation. The rate of FAV-related deaths was 32%. These patients underwent FAV earlier than live-born fetuses (median GA at FAV 22 weeks [range, 20.0-25.0] vs. 24.5 weeks [range, 21.0-32.0], respectively, p = 0.031). Conclusions: A significant proportion of fetuses with CAS who undergo technically successful FAV have BVC postnatally. However, FAV implies a high risk of fetal death, which highly depends on the GA at which this intervention is required.

Prenatal Prediction of Surgical Approach for Coarctation of the Aorta Repair

Objective: To evaluate the capacity of fetal echocardiography for predicting the more likely surgical approach in newborns with coarctation of the aorta (CoAo) (left thoracotomy vs. median sternotomy). Material and Methods: We selected all cases of suspected CoAo prenatally diagnosed in 2003-2012 (n = 95). 49/95 were considered at high-risk and 46/95 at low-risk of CoAo, and 38/49 and 7/46 were postnatally confirmed, respectively. We firstly evaluated in 40 cases of CoAo surgically repaired (24 thoracotomy, 16 sternotomy) whether there were differences in fetal echocardiographic parameters between both groups. Secondly, we assessed the performance of these parameters for predicting the surgical approach in fetuses at high risk of CoAo. Results: Sternotomy approach was associated with higher rate of postoperative complications and longer hospital stay compared with thoracotomy (81.3 vs. 41.7%, p = 0.014; 30.5 vs. 15.4 days, p = 0.0004, respectively). The Z-score of the aortic isthmus, measured in the sagittal plane, was significantly smaller in the sternotomy group. In fetuses at high-risk of having CoAo a cut-off value of the isthmus Z-score ≤ -2.5 had sensitivity of 78% and specificity of 82% for predicting sternotomy approach, with 9% false positive cases in which CoAo was not confirmed. Discussion: The surgical approach for CoAo repair may be prenatally predicted by means of the Z-score of aortic isthmus, measured in the sagittal plane.

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.