A 25-year-old healthy primigravida, who had a consanguineous partner, was referred for fetal echocardiography at the 22nd week of gestation. Based on an abnormal three-vessel view at the 20 weeks anomaly scan in a first-level center, a non-specified congenital heart defect was suspected. First-trimester prenatal screening (nuchal translucency measurement) was not performed. The family history was negative for congenital heart defects.Echocardiography revealed visceroatrial situs solitus with normal systemic and pulmonary venous connections and normal intracardiac anatomy. The ascending aorta had a normal size (3.7 mm), however, a right aortic arch (RAA) was present. A left patent ductus arteriosus (PDA) was running from the brachiocephalic artery connecting end-to-end with the left pulmonary artery (LPA) (Figure 1a and b). The usual pulmonary arterial bifurcation was not present. The main PA had a normal size (4.8 mm) and continued into the right PA (RPA), but was not connected to the LPA (Figure 2). A right-sided PDA connected the right RPA with the RAA. Amniocentesis for karyotyping and analysis of 22q11 deletion was offered but declined by the parents. Follow-up scans supported the suspected diagnosis.At 37 + 3 weeks, a boy of 3760 g was born after an uncomplicated delivery. The physical examination and arterial oxygen saturation were normal. Echocardiography confirmed the diagnosis of non-confluent pulmonary arteries, distal ductal origin of the LPA from the long left-sided PDA running from the brachiocephalic artery, and a RAA with a right-sided PDA. Genetic testing did not disclose any abnormalities; among others, a possible 22q11 microdeletion was excluded. On day 3, echocardiography demonstrated constriction of both right-sided and left-sided PDA. To prevent occlusion of the LPA, prostaglandin E1 infusion was started, leading to

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Objectives: Microcephaly is reported in 10% of the neonates with hypoplastic left heart syndrome (HLHS). Recent studies suggest that altered intra-uterine brain oxygenation in these defects partly explains these findings. Small prenatal series of head growth in various congenital heart defects (CHD) show varying results. We evaluated the fetal head growth in a large cohort of cases with CHD and investigated the association with the type of CHD. Methods: In a prospectively registered prenatal database of three fetal medicine units, cases with isolated CHD were selected over a 10 year period. Subgroups were made of CHD with compromised oxygen delivery to the brain (eg HLHS), mixed oxygen delivery (atrioseptal defects/conotruncal abnormalities) and normal oxygen delivery to the brain (eg tricupid atresia). The data of head circumference (HC) measurements at different gestational ages were evaluated. Results: Isolated CHD were identified in 525 fetuses; 372 (71%) were liveborn, in 131 (25%) the pregnancy was terminated, in 22 (4%) an intra-uterine demise occured. To date we analysed 230 cases. At 21 (±1,27) weeks the Z-score of the HC was −0,02 (±1,20; n.s.), with a non-significant decrease to −0,25 (±1,08) in the third trimester (mean GA 35,1 (±1,84) weeks). The largest decrease was found in transposition of the great arteries (TGA; n = 16), showing a non-significant decrease from +0,12 (±0,89) at 21 weeks to −0,45 (±0,98) in the third trimester. In left ventricle outflow tract abnormalities (LVOT; n = 59) the Z-score showed a non-significant decrease from -0,16 (±1,19) to -0,37 (±0,85). Other subgroup analyses also did not show significant differences from normals. Conclusions: In contrast to previous observations in smaller studies, this large cohort did not show a significant smaller head circumference at mid-gestation, nor a significant head growth restriction in third trimester among fetuses with isolated CHD. Only fetuses with TGA and LVOT show a slight, but non significant decrease in HC. OP01.02Diagnostic accuracy of prenatal echocardiography in congenital heart disease C. van Objectives: Prenatal detection of congenital heart defects (CHD) reduces neonatal mortality and morbidity. The accuracy of the prenatal diagnosis of CHD is a critical factor to determine the urgency of immediate postnatal treatment, to predict the course of (surgical) treatment and to enable parents to make an informed decision. This study examines the anatomic accuracy of the prenatal diagnosis of fetuses with a CHD in a large cohort referred to a tertiary care center. Methods:A multicenter retrospective study of third-level ultrasonography referrals between January 2002 and January 2012 was conducted. Prenatal and postnatal diagnoses were compared and the degree of agreement was determined; correct (the postnatal diagnosis led to a similar intervention or outcome as expected), minor discrepancy (the severity of the defect was correctly diagnosed, but required a different surgical intervention than predicted) or no similarity (the ...

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Oral communication abstractsResults: A total of 444 ultrasound evaluations were performed on 119 CHD fetuses, with a median of 2 measurements per fetus. CHD fetuses showed a small head at diagnosis (BPD −1.32 ± 0.99 z scores, HC −0.79 ± 1.02 z-scores), maintained throughout gestation (figure a). UtA and UA Doppler showed an increase towards the end of pregnancy, whereas no changes were observed in MCA or cerebroplacental ratio (CPR) with gestational age (figure b). Conclusions: CHD fetuses showed smaller head biometries since the second trimester of pregnancy, suggesting an early insult that persists throughout gestation. UtA and UA Doppler resistance increased, which may indicate co-existing placental impairment in CHD cases.Supporting information can be found in the online version of this abstract