1/14. Endoscopic ligation of umbilical cord at 19 week's gestation in monoamniotic monochorionic twins discordant for hypoplastic left heart syndrome.We report the first attempt of reduction of monoamniotic twins, discordant for hypoplastic left heart syndrome, using a new fetoscopic technique. Employing sonographic guidance and endoscopic visualization, cord ligation was accomplished, but significant cord entanglement, not previously appreciated, resulted in the ligation of the umbilical cord of the normal fetus. Cord entanglement may frustrate endoscopic techniques in monoamniotic twins.- - - - - - - - - - ranking = 1keywords = gestation (Clic here for more details about this article) |
2/14. prenatal diagnosis of ventriculocoronary arterial fistula in a fetus with hypoplastic left heart syndrome and aortic atresia.In a fetus with a hypoplastic left heart syndrome detected at 26 weeks' gestation, a communication between the left ventricular cavity and the left coronary artery was detected using color Doppler. Bidirectional flow was demonstrated and the ventricle was shown to be filled via the fistula during diastole. After premature birth at 31 weeks and neonatal death, autopsy confirmed the findings. Because the association of a heart defect with ventriculocoronary communications worsens the neonatal prognosis, it should be ruled out in fetuses with hypoplastic left heart syndrome before parents are counseled.- - - - - - - - - - ranking = 0.25keywords = gestation (Clic here for more details about this article) |
3/14. prenatal diagnosis of partial anomalous pulmonary venous connection by detection of dilatation of superior vena cava in hypoplastic left heart. A case report.A 27-week fetus was given targeted fetal echocardiography due to an abnormal four-chamber view. A complex heart malformation was seen with two atria, a single ventricle, and aortic and mitral atresia. In addition to intracardiac findings, a dilated superior vena cava (SVC) was seen, suggesting a possible abnormal pulmonary venous connection to the SVC. An increased venous flow (up to 90 cm/s) was recorded as well. The course of pregnancy was uneventful and the baby was delivered vaginally at term. Cardiac surgery was planned but the baby died on 4th day of life. The autopsy findings confirmed both intracardiac anomalies as well as partial anomalous venous connection to SVC, with no apparent obstruction. This is the first report which stresses that dilatation of the fetal SVC may be suspicious for partial abnormal pulmonary venous connection during prenatal life.- - - - - - - - - - ranking = 0.018622943270454keywords = pregnancy (Clic here for more details about this article) |
4/14. Concordance for hypoplastic left heart syndrome in a monochorionic twin pregnancy.The risk of structural heart disease is significantly higher in twin pregnancies than in singleton pregnancies, but the concordance rate has been found to be relatively low, even in monochorionic pregnancies. This is the first report of a monochorionic twin pregnancy concordant for hypoplastic left heart syndrome (HLHS), the diagnosis having been made by fetal echocardiography at 15 weeks' gestation. The findings were confirmed at necropsy at 17 weeks' gestation, following termination of pregnancy. Both twins had mitral and aortic atresia, with severely hypoplastic aortic arches. This report adds weight to there being a genetic component to the cause of HLHS in some cases and illustrates how the findings from early fetal echocardiography with postmortem follow up can help to extend the understanding of the aetiology of this condition.- - - - - - - - - - ranking = 0.61173765962272keywords = gestation, pregnancy (Clic here for more details about this article) |
5/14. prenatal diagnosis of hypoplastic left heart syndrome and trisomy 18 in a fetus with normal nuchal translucency and abnormal ductus venosus blood flow at 13 weeks of gestation.We describe a case of early prenatal diagnosis of a major congenital heart anomaly and trisomy 18 in a low-risk pregnant woman. Nuchal translucency (NT) measurement at 13 weeks' gestation was 1.2 mm and Doppler evaluation of the ductus venosus detected a persistent reversed flow during atrial contraction. This finding prompted us to perform fetal echocardiography which showed hypoplastic left heart syndrome. karyotyping following chorionic villus sampling diagnosed trisomy 18. review of the recent literature suggests that the finding of an abnormal ductus venosus Doppler pattern in the late first trimester of pregnancy may be an early sign of either congenital cardiac or chromosomal abnormality, even in the presence of normal NT screening.- - - - - - - - - - ranking = 1.2686229432705keywords = gestation, pregnancy (Clic here for more details about this article) |
6/14. Diminutive fetal left ventricle at mid-gestation associated with persistent left superior vena cava and coronary sinus dilatation.In a fetus with a small left ventricle diagnosed at mid-gestation, a persistent left superior vena cava connected to a dilated coronary sinus was detected. Although the size of the mitral annulus appeared to be normal, opening of the mitral valves was restricted in diastole. Echocardiographic follow-up showed no significant growth of the left ventricle and termination of pregnancy was carried out at 31 weeks at the parents' request. In addition to the prenatal findings, postmortem examination revealed a small mitral annulus with abnormal insertion of the mitral valve chordae tendineae. We hypothesized that abnormal venous return to a dilated coronary sinus may have led to mitral valve dysfunction and hypoplasia. On the other hand the left ventricular hypoplasia we observed may have involved a global abnormality of the left-sided cardiac structures.- - - - - - - - - - ranking = 1.2686229432705keywords = gestation, pregnancy (Clic here for more details about this article) |
7/14. hypoplastic left heart syndrome associated with left ventricle-coronary artery-pulmonary artery fistula: prenatal diagnosis and histological correlation.We describe an unusual case of left ventricle-left coronary artery-pulmonary artery fistula associated with hypoplastic left heart syndrome (HLHS) and a ventricular septal defect, in which findings on echocardiography and histological analysis were correlated. The diagnosis was made at 22 weeks of gestation. We speculated that the presence of the fistula was the primary cause of the HLHS due to the existence of the ventricular septal defect that nullified the intraventricular pressure gradient, which is typical of hypoplastic ventricles and thought to be responsible for the secondary development of fistulas in other cases.- - - - - - - - - - ranking = 0.25keywords = gestation (Clic here for more details about this article) |
8/14. In utero cardiac fetal surgery: laser atrial septotomy in the treatment of hypoplastic left heart syndrome with intact atrial septum.OBJECTIVE: The purpose of this study was to report a novel technique, laser atrial septotomy, for the in utero treatment of hypoplastic left heart syndrome with intact atrial septum. STUDY DESIGN: In utero atrial septotomy by neodymium-YAG laser photofulguration in a fetus with hypoplastic left heart syndrome (HLHS) and intact atrial septum was performed at 30 4/7 weeks of gestation. Percutaneous fetal cardiocentesis was performed to guide a contact (neodymium-YAG) laser fiber into the right atrium with the objective of creating an interatrial communication by photofulguration of the septal tissue. RESULTS: New onset of blood flow from the left to the right atrium was confirmed by color Doppler imaging during the procedure. The neoatrial septal defect remained patent until delivery. A 3400-g neonate was born by spontaneous vaginal delivery at 37 weeks of gestation. A first stage Norwood procedure was performed on the first day of life and surgical correction of an obstructed right pulmonary vein at 3 months. Although pulmonary vascular resistance was normal at cardiac catheterization at 2 months of age, the infant died at 5 months of age from multiple organ failure. An autopsy was declined. CONCLUSION: In utero laser atrial septotomy is feasible. Further experience is necessary to determine the risks and benefits of this technique for the treatment of fetuses hypoplastic left heart syndrome with intact atrial septum.- - - - - - - - - - ranking = 0.5keywords = gestation (Clic here for more details about this article) |
9/14. Pseudotrisomy 13 syndrome: a case with left ventricular hypoplasia and duodenal stenosis.We report a case of a female child born at 32 weeks of gestation. birth weight was 1200 g (<3rd centile), length 40 cm (10th-50th centile) and head circumference 23.5 cm (<3rd centile). Clinical examination revealed microcephaly, hypotelorism, microphthalmia, a flat rudimentary nose with a single nasal cavity, high palate, thick dysplastic low-set ears, a short neck, postaxial polydactyly of the upper limbs, and single palmar creases. Investigations showed alobar holoprosencephaly, absence of the third ventricle and midline structures of the brain, microphthalmia, hypotelorism, left ventricular hypoplasia, a large atrial septal defect, and duodenal stenosis. The karyotype was 46,XX. A hypoplastic left ventricle and duodenal stenosis have not been previously reported in pseudotrisomy 13 and this case might aid in the further delineation of this syndrome.- - - - - - - - - - ranking = 0.25keywords = gestation (Clic here for more details about this article) |
10/14. prenatal diagnosis of ventriculocoronary arterial communication in fetuses with hypoplastic left heart syndrome.OBJECTIVE: The purpose of this series was to describe the fetal echocardiographic findings in hypoplastic left heart syndrome with aortic atresia and ventriculocoronary arterial communication and implications of these findings. methods: We describe 2 fetuses with hypoplastic left heart syndrome with ventriculocoronary arterial communication diagnosed at 29 and 20 weeks' gestation, respectively. The underlying cardiac anatomy consisted of a hypoplastic left heart and mitral stenosis with aortic atresia. We used color Doppler and pulsed Doppler sonography on the surface of the myocardium to specifically look for coronary arterial flow. RESULTS: By color Doppler sonography, ventriculocoronary arterial communication was shown between the left ventricular cavity and the left coronary artery with characteristic bidirectional flow on pulsed Doppler examination. There was no mitral regurgitation. The left ventricular myocardium was substantially hypertrophied. The first patient underwent surgical Norwood palliation and died after a prolonged postoperative course. The second patient underwent stenting of the arterial duct and bilateral pulmonary artery banding in the catheterization laboratory but died after a few weeks. Implications of ventriculocoronary arterial communication in association with hypoplastic left heart syndrome are discussed. CONCLUSIONS: It is possible to accurately diagnose ventriculocoronary arterial communication on fetal echocardiography. The presence of ventriculocoronary arterial communication is seen exclusively in a subgroup of patients with an aortic atresia and mitral stenosis variant of hypoplastic left heart syndrome. The prognosis is poor in this subgroup of patients.- - - - - - - - - - ranking = 0.25keywords = gestation (Clic here for more details about this article) |
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