Oct 7, 2007 - died after repair. 8. 33. DORV, VSD, Situs inversus totalis, ... as it manifests late in pregnancy, usually after 32 weeks' GA. Few cases have been ...
7–11 October 2007, Florence, Italy
Poster abstracts
P39.08: Table Gestational week
1
38
2
31
3
34
4
32
5
31
6 7 8
36 39 33
9 10 11
29 28 35
12 13
21 26
14
28
15 16 17 18
24 34 23 36
19
24
Fetal diagnosis
Postnatal diagnosis
Karyotype
Extracardiac abnormalities
Outcome
DORV, VSD, mitral atresia, aorta stenosis, hypoplastic left heart DORV, VSD, single AV valv, pulmonary stenosis DORV, VSD, ASD
DORV, VSD, mitral atresia, c-TGA, Pulmonary stenosis, hypoplastic left heart not available; TOP
not done
not found
died after repair
trisomy 18
vermis hypoplasia, IUGR
TOP
DORV, VSD, coarctation of aorta, Taussing-Bind DORV, VSD, tricuspid atresia, hypoplastic right ventricle DORV, AVSD, pulmonary stenosis
not done
oligohydroamnios, ARED, preeclampsia not found
died after repair
trisomy 18
lost in follow-up DORV, VSD lost in follow-up
DORV, VSD, tricuspid stenosis, aorta stenosis, hypoplastic right ventricle DORV, AVSD, pulmonary stenosis DORV, VSD, pulmonary stenosis DORV, VSD, Dextrocardia DORV, VSD, Situs inversus totalis, bradycardia, v. azygous, systemic venous anomaly Twin B; DORV, AVSD, hypoplastic left heart DORV, VSD, pulmonary stenosis DORV, AVSD, single AV valve, hypoplastic left ventricle, pulmonary stenosis DORV, VSD, aorta stenosis DORV, VSD, mitral atresia, coarctaton of aorta, hypoplastic left ventricle, tricuspid regurgitation DORV, VSD, mitral atresia, hypoplastic left heart, endocardial fibroelastosis DORV, VSD,, pulmonary stenosis DORV, VSD DORV, single AV valve, hypoplastic left heart DORV, AVSD, hypoplastic left heart, pulmonary stenosis DORV, mitral atresia, hypoplastic left heart
not done
alive
not done not done not done
Dandy Walker, bilateral pelvicaliectasis single umbilical artery single umbilical artery Situs inversus totalis
neonatal death lost in follow-up died after repair lost in follow-up
DORV, AVSD, hypoplastic left heart not available; TOP DORV, AVSD, single AV valve, hypoplastic left ventricle, pulmonary stenosis DORV, VSD; TOP not available; TOP
not done not done not done
not found ensephalocel not found
neonatal death TOP neonatal death
trisomy 21 46**
bilateral pelvicaliectasis not found
TOP TOP
DORV, VSD, tricuspit atresia, single functional ventricle DORV, VSD, pulmonary atresia DORV, VSD not available; TOP DORV, AVSD, hypoplastic left heart
46**
not found
neonatal death
46** 46** not done not done
oesophageal atresia single umbilical artery not found IUGR
alive died after repair TOP neonatal death
DORV, mitral atresia; TOP
46**
not found
TOP
ventricle showed some hypertrophy. There was no evidence for an aortico-left ventricular tunnel. Aortic regurgitation is a serious lesion in the fetus producing chronic volume overload and fetal hydrops. It may be associated aortic stenosis and can lead to severe left ventricular hypertrophy, ventricular dilation and myocardial fibrosis. Postnatally the systemic vascular resistance increases, aggravating the aortic insufficiency with detrimental effects. When nuchal edema persists in the second trimester there is an increased risk of an unspecified genetic syndrome, often with an associated cardiac defect. The nuchal edema may have been related to cardiac dysfunction or have been a feature of an unspecified genetic syndrome.
Results: Three fetuses presented with a circular anechogenic vascular structure of 11 mm of diameter, as a continuation of the pulmonary artery in the three vessels’ view at 30 to 32 weeks. No drugs were used during pregnancy. Follow-up scans did not show a progression in size. One case had an IUGR and the other two were > p90. Postnatally, the first case could not be seen by echocardiography but confirmed by angio-CT, with spontaneous resolution at two months. The second case was found to have an aorto-pulmonary collateral artery. The third patient was followed by echocardiography and spontaneous resolution occurred in the first days. Conclusion: Aneurisms of the ductus arteriosus may be a more frequent condition than previously expected; development in late pregnancy and early spontaneous resolution may mis-diagnose some patients. It is unclear whether this condition is a cardiovascular malformation or an anatomical variation but it should be considered in presence of IUGR or in macrosomic fetuses. Early angio-CT may help to confirm the diagnosis in order to assess the impact on morbidity and mortality.
P39.10 Antenatal diagnosis of aneurisms of the ductus arteriosus and postnatal confirmation by angio-CT
P39.11 Signs of cardiac overload: a hint to look for angiomatous malformations
P. Valentini, L. A. Caicedo, A. Insunza, M. Yamamoto
H. Delier1 , I. Kalelioglu1 , R. E. Omeroglu2 , H. Kayserili3 , R. Has1
Hospital Padre Hurtado y Clinica Alemana, UDD, Chile Introduction: Isolated ductal aneurisms are rare (1/1000 liveborns) and resolve spontaneously in most cases. In utero diagnosis is rare as it manifests late in pregnancy, usually after 32 weeks’ GA. Few cases have been reported with postnatal confirmation. Indeed, it is unclear whether it could be an anatomical variation or a fetal functional abnormality with postnatal resolution. Objectives: To report three cases suspected at our institutions, with postnatal 1-year follow-up. Neonatal angio-CT was performed in two and echocardiography in all cases. Methods: Retrospective collection of prenatal ultrasounds and postnatal follow-up.
Ultrasound in Obstetrics & Gynecology 2007; 30: 547–653
1 Istanbul Faculty of Medicine, Department of Obstetrics and Gynecology, Turkey, 2 Istanbul Faculty of Medicine, Department of Pediatric Cardiology, Turkey, 3 Istanbul Faculty of Medicine, Department of Medical Genetics, Turkey
We describe a case with prenatal findings suggestive of coarctation of the aorta and diagnosed to have an angiomatous malformation causing cardiac overload postnatally. A 36-year-old, G2, P1 woman was referred at 30 weeks of gestation due to cardiomegaly. Sonographic examination revealed cardiomegaly and discrepancy in the sizes of the ventricles. Right ventricle and pulmonary artery were enlarged in comparison to the left ventricle and aorta.
599
17th World Congress on Ultrasound in Obstetrics and Gynecology
Postvalvular dilatation was detected in the main pulmonary artery. The brachiocephalic trunk was enlarged relative to the aortic arch and color Doppler imaging revealed reverse flow in the aortic arch. Therefore, coarctation of the aorta was suspected. No other structural anomalies were detected. Karyotype analysis was declined. Follow-up scans were unremarkable. A female infant weighing 2910 g was delivered by Cesarean section at 39 weeks. The right arm, forearm and hand of the baby were enlarged with purple-red discoloration. Postnatal echocardiography did not confirm coarctation of the aorta. Doppler sonography, MR and CT angiography revealed dilated and tortuous brachiocephalic trunk, right subclavian, axillary and brachial arteries with diffuse collateral vessels. Right cephalic vein was also dilated while right brachial, axillary and subclavian veins were normal. No accompanying mass lesions were detected. Klippel-Trenaunay-Weber syndrome was diagnosed due to the macular vascular nevus, arteriovenous malformation and related hypertrophy of the bony structures. Interventional treatment was not recommended for the arteriovenous malformation. The baby was discharged on the 35th day, with digoxin and furosemide treatment. At present, the baby is two years old and doing well on digoxin and captopril therapy. In conclusion, prenatal detection of high cardiac output state should prompt a detailed, targeted examination for angiomatous malformations with careful examination of the limbs.
P39.12 Giant sacrococcygeal teratoma with normal cardiac function I. Bianchi, D. C. Wood, S. Weiner, J. Baxter, V. Berghella Thomas Jefferson University, United States Ultrasound diagnosis of a giant sacrococcageal teratoma (SCT) is typically made after 24 weeks’ gestation. Giant SCT is defined as the tumor being greater in circumference than the fetal head and carries a poor prognosis. Large or giant SCTs are typically associated with arterio-venous malformations (AVM) within the tumor and with signs of fetal congestive heart failure (CHF) from increased cardiac output and may be associated with hydrops and polyhydramnios. We present a case of a fetus with giant SCT where the tumor was greater in size than the fetal trunk (23 × 10 cm; 800 g) yet there were no signs of either AVM or CHF. The first ultrasound identifying the SCT was at 26 weeks (8 × 10 cm) at the referring hospital. The mother was transferred to our center for pre-eclampsia at 28 weeks. At 29 weeks, the fetus developed mildly elevated umbilical artery Doppler pulsatility index (PI 1.8) along with placentomegaly and polyhydramnios. During these 3 weeks, the tumor more than doubled in size. The mother developed premature rupture of membranes and the infant was delivered by Cesarean section at 31 weeks’ gestation at 2190 g and with Apgar scores of 8 and 9. The child underwent staged bulk reduction at 1 day and closure modification at 1 week. There were no malignant cells found within the tumor at pathology. The child was discharged home at 64 days. In this case the cardio-thoracic ratio was normal, and there were no abnormal Doppler findings within the fetus, nor any abnormal fluid accumulations inside the fetus. There was no AV malformation within the SCT by either pulsed or color Doppler. This SCT had grown precipitously without CHF. As the fetus was delivered at 31 weeks we can not say that CHF with hydrops would not have developed at a later gestational age.
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Poster abstracts
P39.13 Can the hyperoxygenation test predict neonatal pulmonary function after preterm birth? D. C. Wood, J. C. Sabogal, J. Baxter, S. Weiner, V. Berghella Thomas Jefferson University, United States Preterm birth resulting from pre-eclampsia, severe fetal grow restriction and premature rupture of the membranes occurs in the USA in 10% of live births. Complications include neonatal respiratory disease associated with lung immaturity. Despite the use of antenatal steroids and postnatal surfactant therapy, a large percentage of these preterm infants will have continuing pulmonary complications. The testing for lung maturity remains necessary. Currently, prediction of lung maturity is done by evaluating the lecithin to sphingomyelin ratio and by identifying the presence of phosphatidylglycerol in amniotic fluid, requiring the performance of an amniocentesis. The ratio can be affected by maternal diabetes while the assay may be corrupted by the presence of either fetal blood or meconium in the sample. We have shown that the non-invasive hyperoxygenation test, performed during fetal echocardiography, assesses the pulmonary vascular response to oxygen after 30 weeks’ gestation in normal and abnormal fetal conditions. A positive change shows increased Doppler pulmonary flow patterns in the branch pulmonary arteries after maternal hyperoxygenation as compared to the mother breathing room air. In a preliminary study, we have attempted to develop a rapid and reliable method of predicting neonatal pulmonary function using the hyperoxygenation test in the labor and delivery suite. We questioned whether a normal or abnormal response would correlate with the neonatal respiratory outcomes, regardless of gestational age or whether the mother received antenatal steroids. In addition, we sought to establish whether antenatal steroids have any effect on Doppler flow patterns. If reliable, this hyperoxygenation test could be useful in planning therapies in the critical newborn period.
P39.14 The ductus venosus is responsive to maternal hyperoxygenation in fetuses with severe growth restriction D. C. Wood, J. C. Sabogal, I. Bianchi, J. Baxter, S. Weiner, V. Berghella Thomas Jefferson University, United States Fetuses with significant growth restriction exhibit abnormal Doppler pulsatility findings, first in the free loop portion of the umbilical artery (FLUA), then in the middle cerebral artery (MCA) and finally in the ductus venosus (DV) with pulsations in the intraabdominal umbilical vein just prior to the development of hydrops fetalis or fetal demise. Therapies intended to postpone delivery include maternal bed rest and an increased maternal oxygen environment. Although in milder cases the blood flow in the FLUA and MCA may return toward normal Doppler flow patterns with therapy, in more severe cases, the abnormal pulsatility will persist. In both settings, Doppler pulsatility in the DV may measure within normal limits, but the size of the DV is greater than the normal range. The maternal hyperoxygenation test (MHT) during fetal echocardiography was applied to a group of women who were hospitalized for preeclampsia, premature rupture of membranes and severe fetal growth restriction (SFGR). The Doppler blood flow measurements were acquired in room air and in 60% oxygen. Although this test was used to evaluate changes in pulmonary flow patterns in fetuses, here we applied the MHT to compare the Doppler flow patterns in the FLUA, MCA and DV and the diameter at the narrowest portion along the length of the DV. Fetuses with SFGR associated with low amniotic fluid have redistribution of cardiac output toward the brain, coronary and adrenal circulations, and away from the kidneys and the lungs. The DV appears to dilate in diameter yet the DV Doppler flow pattern may remain normal as the as the FLUA and MCA become more abnormal. During the MHT, the FLUA
Ultrasound in Obstetrics & Gynecology 2007; 30: 547–653
