M. Respondek-Liberska

F131The role of fetal echocardiography during maternal pharmacological treatment

Background

P26.05: Sonographic and echocardiographic features in fetuses with meconium peritonitis and ileus

method. These parameters were compared to age matched normal fetuses from our data base. Results: We reviewed echocardiograms of 31 fetuses with isolated GSC. None of the fetuses had structural heart defects consistent with congenital heart disease. There were no functional abnormalities. There was no statistical difference between the GSC group and normal fetuses by measurement of our FECHO parameters. In five (16%) fetuses, we identified left axis shift of 45 to 75 degrees off the sternum to spine axis within the left chest. Conclusions: Structural and functional cardiac abnormalities are rare in fetuses with isolated GSC. The axis shift of the heart within the thorax is likely associated with amount of bowel that is outside the abdomen. This axis shift is also seen with omphalocele where the larger the omphalocele the greater the shift.

P26.22: Sonography in fetal ovarian cysts

routine ultrasound. A bilateral renal disease with oligohydramnios was diagnosed in her first pregnancy and the baby was stillborn with Potter sequence. In the actual pregnancy the ultrasound done at 26 + 4 weeks of gestation showed right pyelocalicial and proximal ureteral dilatation with narrowing. In the neonatal period a 3D scan with multiplanar views demonstrated a megaureter associated with ureteral valve and a distal ureteral stenosis. The diagnosis was confirmed via surgical exploration and histology. While rare this might be a cause of ureteral obstruction with progressive upper tract dilatation. We emphasize the value of multiplanar 3D which allowed a diagnosis that only few years ago was privative of excretory urography.

P39.02: Fetal echocardiography in ovarian cysts

ranged from 2 to 36 (mean, 18.87 ± 13.07) months. In most cases the tumor masses decreased after birth (n = 6), but had no change in utero (n = 5). Three of them were diagnosed as tuberous sclerosis after birth, and none of them needed surgical intervention. Conclusion: Fetal cardiac tumors and their effect on the fetal cardiac function could be well evaluated by two-dimensional and Doppler echocardiography. The fetal cardiac tumor may have little effect on the fetal well being, both prenatally and postnatally. We cannot suggest the definite association of fetal rhabdomyomas and tuberous sclerosis.

OP02.08: Non immune hydrops fetalis (270 cases)—optimization of diagnosis and therapy

our series of fetuses with pleural effusion treated with thoracoamniotic shunting. Methods: Between 1992 and 2005, 53 fetuses with pleural effusion were referred to our unit and underwent thoraco-amniotic shunting. In all cases a detailed fetal anatomy scan and karyotyping were performed. A search for maternal infection was done in every case. A double pigtail catheter (Rocket, London, UK) was inserted by one operator under ultrasound guidance using local anesthesia when a complication like hydrops or polyhydramnios was found. Results: There were 35 bilateral and 18 unilateral shunt placements. In all 53 cases the procedure was successful. In two cases there was termination of pregnancy because of abnormal karyotype. There were 12 neonatal deaths. There were 34 survivors, with a follow-up at 1–7 years for 45% of cases. Survival rate was 90% for fetuses without hydrops and 58% for those which were hydropic, compared with an overall survival rate of 59% and 35%, respectively, as reported in untreated cases. Conclusions: Thoraco-amniotic shunt may improve outcome for fetuses with pleural effusion expecially if complicated with hydrops.

OP06.08: Prenatal constriction of ductus arteriosus—analysis of 30 cases

in one of the six cases, the only case where DAA was associated with another heart defect. As to pregnancy outcome, there was one termination of pregnancy. Of the five newborns, three required surgery and two remain asymptomatic at over 6 months of age. Conclusions: The anomaly most likely to cause a symptomatic vascular ring is DAA. Fetal echocardiography appears to reliably diagnose DAA albeit with some limitations in the thorough assessment of the pathology. Establishment of 22q11 microdeletion status in those cases with associated cardiac anomalies is important.

OP04.01: Pulmonary venous blood flow velocities in fetal hypoplastic left heart syndrome (HLHS)

Objective: Rhomboencephalosynapsis (RES) is a rare, but increasingly recognized malformation consisting of fused cerebellar hemispheres, dentate nuclei and superior cerebellar peduncles and absent vermis. RES is associated with significant neurocognitive delay, seizures, and movement disorder. Associated central nervous system abnormalities are common. We report five fetal cases and discuss the fetal ultrasound and magnetic resonance imaging (MR) findings. All cases presented with hydrocephalus. Methods: Institutional ethics approval was obtained. Prenatal ultrasound is available in five cases, prenatal MR imaging in two, postnatal ultrasound and MR in two and autopsy confirmation in three. Images were evaluated for presence and degree of vermian fusion, presence of primary fissure and fastigial point and associated central nervous system anomalies. Results: Fetal and postnatal MR readily identified the classic features of hypoplastic single lobed cerebellum with transverse folia and associated intracranial malformations, specifically within the realm of holoprosencephaly. Both also identified diamond shaped 4th ventricle on axial views, rounded ‘fastigial point’ and lack of primary fissure. Postnatal MR more readily identified fusion of the dentate nuclei. Fetal and postnatal ultrasound documented the transverse folia and narrow transverse diameter of the cerebellum. Conclusion: Rhomboencephalosynapsis is a rare lesion that is best imaged prenatally with fetal MR. Suspicious ultrasound features are cerebellar hypoplasia and absence of the vermis. The presence of these ultrasound findings should raise the clinical suspicion of RES, and be an indication for fetal MR if available.

No increase in peak systolic velocity of the pulmonary veins over time: a potential marker for congenital lobar emphysema.

9. Malone FD, Ball RH, Nyberg DA, Comstock CH, Saade G, Berkowitz RL, Gross SJ, Dugoff L, Craigo SD, Timor IE, Carr SR, Wolfe HM, Trip T, D’Alton ME. First trimester nasal bone evaluation for aneuploidy in an unselected general population: results from the FASTER trial. Am J Obstet Gynecol 2003; 189: S79. 10. Lee W, DeVore GR, Comstock CH, Kalache KD, McNie B, Chaiworapongsa T, Conoscenti G, Treadwell MC, Johnson A, Huang R, Romero R. Nasal bone evaluation in fetuses with Down syndrome during the second and third trimesters of pregnancy. J Ultrasound Med 2003; 22: 55–60.

P01.31: Prenatal ductal constriction (DC) and prenatal foramen ovale (FO) closure in otherwise normal heart anatomy in referral center for fetal echocardiography

anomalies in both groups was similar (81% in left isomerism versus 100% in right isomerism). The most frequent cardiac anomalies observed in left isomerism were ventricular septal defect (6/21; 28.6%), complete atrioventricular septal defect (4/21; 19%) and double outlet right ventricle (3/21; 14.3%). The most frequent cardiac defects in right isomerism were double outlet right ventricle (8/15; 53.3%) and complete atrioventricular septal defect (7/15; 46.7%). Bradyarrhythmia was a notable feature of left isomerism (8/21; 38%). Conclusion: Although both forms of heterotaxy syndrome have probably the same origin, affected fetuses exhibit distinct spectrum of cardiac anomalies. Our study confirms that fetuses with right isomerism tend to have more complex cardiac defects while fetuses with left isomerism are likely to have bradyarrhythmia. Prenatal distinction between left and right is therefore mandatory for optimized parental counseling.

OC028: Natural history of fetal Ebstein's anomaly in a referral center in the second half of pregnancy

In postnatal life progression of heart disease has been clearly documented. Natural history studies have been performed and as a consequence, guidelines for interventions (drugs, interventional or surgical procedures) have been developed. Early detection of fetal heart disease, even in the first trimester, is feasible and these fetuses can be followed in-utero for months. Progression of fetal congenital heart disease has been widely documented in case reports and small case series. Any cardiac valve can become more stenotic, even atretic or more incompetent during the course of pregancy, potentially leading to secondary damage to the heart and lungs. Consecutive impaired growth of ventricles and/or vessels may result in a univentricular circulation postnatally. Valve regurgitations have the potential to lead to hypertrophy, congestive heart failure and hydrops. Theoretically, as in postnatal life, timely fetal intracardiac intervention should lead to normalization or improvement of flows, pressures and hemodynamics, thus preventing secondary damage to the fetal heart and organs, congestive heart failure or fetal death. Cardiac lesions, that might profit from timely interventions could be subgroups of: critical valvar aortic stenosis, critical valvar pulmonary stenosis, pulmonary atresia with intact septum or a closed or severely restrictive foramen ovale in cases of left heart obstructive lesions like e.g. hypoplastic left heart syndrome. Successful in-utero dilatation of aortic and pulmonary valve, perforation of valvar pulmonary atresia and atrio-septostomy with ballon dilation of the created ASD have already been reported. As these procedures carry a high risk of technical problems, morbidity and mortality, careful patient selection is crucial.