The fetal venous system, Part II: ultrasound evaluation of the fetus with congenital venous system malformation or developing circulatory compromise

Yagel, Simcha; Kivilevitch, Z.; Cohen, S. M.; Valsky, D. V.; Messing, B.; Shen, O.; Achiron, Reuven

doi:10.1002/uog.7622

Cited by 108 publications

(126 citation statements)

References 135 publications

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“…This may be to the left or the right sided atrium, or to both, and the hepatic veins connect separately to the heart if the suprarenal inferior cava is present. This venous connection was present in all patients with left isomerism (6). The majority of systemic venous anomalies are accounted for by a few specific abnormalities.…”

Section: Discussionmentioning

confidence: 71%

The Anomalies of Systemic Venous Connections in Children with Congenital Heart Disease

et al. 2014

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Section: Discussionmentioning

confidence: 71%

The Anomalies of Systemic Venous Connections in Children with Congenital Heart Disease

et al. 2014

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“…Un shunt grueso, del calibre de la umbilical, se asocia según estos mismos autores con peor evolución (Figura 4). Para algunos autores, un factor pronóstico relevante es la localización del drenaje de la VU, ya que aquellos casos en los que no hay by pass hepático tienen mucha mejor evolución (7). Es importante reseñar la enorme dificultad que entraña en ocasiones demostrar la presencia de shunt portosistémico (Figura 5).…”

Section: Discussionunclassified

“…Es una anomalía rara y de difícil diagnóstico, aunque algunas series publicadas por grupos muy expertos y en población de alto riesgo lo describen en hasta 6/1000 exploraciones (7). Dos rutas diferentes para el retorno de la VU han sido descritos en estos fetos; drenaje extrahepático o shunt portosistémico (conexión directa de la VU a la vena iliaca, a la cava inferior (VCI), a la aurícula derecha (AD), a las venas suprahepáticas o incluso al seno coronario) (5-8) o drenaje intrahepático o shunt umbilico hepático (conexión de la VU al seno portal).…”

Section: Introductionunclassified

Diagnóstico prenatal y evolución en fetos con agenesia de ductus venoso

Pérez-Pedregosa

S.R

et al. 2014

Rev. chil. obstet. ginecol.

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RESUMENAntecedentes: El ductus venoso es una derivación vascular (shunt) presente en el feto que permite el paso de sangre oxigenada de la vena umbilical (VU) hacia la circulación coronaria y cerebral. Su agenesia se asocia con defectos cromosómicos, síndromes genéticos, defectos estructurales y complicaciones prenatales como crecimiento intrauterino retardado y muerte fetal. Resultados: Se analizaron 15 agenesias de ductus venoso (ADV) en gestaciones únicas entre enero de 2010 y diciembre de 2013. El 80% de ellas fueron diagnosticadas en la exploración rutinaria de la semana 12. Se realizó estudio de cariotipo en el 53% de los casos (8/15), bien por riesgo alto de cromosomopatía en el cribado combinado y/o translucencia nucal aumentada (75%) o malformaciones asociadas (25%). Sólo hubo un diagnóstico de trisomía 21 y postnatalmente de una microdelección del cromosoma 7. Realizaron interrupción legal del embarazo un total de 4 pacientes (por trisomía 21 o por alteraciones estructurales). Entre las 11 gestaciones restantes un 27% se diagnosticó RCIU, hubo una muerte neonatal a las 12 horas de vida por síndrome de aspiración meconial e hipertensión pulmonar. En un 60% se objetivó la presencia de un drenaje umbilicohepático y entre los 6 restantes con shunt portosistémico, 4 tenían conexión entre la VU y la VCI. Conclusión: La ADV es una anomalía infrecuente del sistema venoso fetal, de difícil diagnóstico y con mal pronóstico en aquellos casos en que se asocia con otros marcadores y/o anomalías estructurales que pueden aparecer tardíamente, por lo que debe realizarse un seguimiento adecuado.PALABRAS CLAVE: Agenesia de ductus venoso, ductus venoso, diagnóstico prenatal, ecografía 3D doppler color, sistema venoso fetal, sistema portal fetal, derivación vascular SUMMARY Background:The ductus venosus (DV) is a unique shunt that allows direct passage of oxygenated blood from the umbilical vein (UV) to the coronary and cerebral circulation by a preferential passage through the foramen ovale. DV agenesis (DVA) is associated with chromosomal abnormalities, genetic syndromes, structural defects and prenatal complications such as intrauterine growth retardation (IUGR) or even stillbirth. Results: We report 15 cases of DVA in singleton pregnancies between January 2010 and December 2013. 80% of them were diagnosed on routine examination during the 11-14 weeks scan. Karyotyping was performed in 53% of cases (8/15) by high risk of chromosomal abnormalities in the first trimester combined screening and/or an increased nuchal translucency thickness in 6/8 (75%), or associated malformations 2/8

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“…Secondary to the physiological diameter reduction of the DV, the blood flow increases significantly. This allows the oxygenated blood to selectively cross the foramen ovale and to drain into the left atrium, to reach the left ventricle and the ascending aorta, and thus the coronary and the brain vessels without being mixed with deoxygenated blood issued from the inferior and the superior vena cava [1][2][3][4][5].…”

Section: Embryological Backgroundmentioning

confidence: 99%

Persistent Right-Sided Umbilical Vein and Azygos Continuity associated with Ophthalmological and Pulmonary Malformations. Prenatal Diagnosis of an Exceptional Case

Lepage

Capelle

Emonts

et al. 2014

Gynecol Obstet (Sunnyvale)

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We report on the case of a fetus in whom a prenatal ultrasound performed at 22 weeks of gestation allowed the diagnosis of a double anomaly of the systemic venous return, with a persistent right umbilical vein and the agenesis of the inferior vena cava with azygos continuity.The fetus showed also a large umbilical hernia and a right sided microphtalmia. Fetal growth was normal. Genetic anomalies were excluded by karyotype and CGH array. Cesarean section was performed at 39 weeks of gestational age for breech presentation. The neonate adapted well. Post-natal examination confirmed pre-natal diagnosis. Thoraco-abdominal CT-scan performed at 8 days of age showed additional right upper lobe bronchial atresia with relative hypoplasia of the right pulmonary artery and right hepatic lobe hypotrophy. It also showed a short segmental infra diaphragmatic aplasia of the vena cava with dilation of the azygos vein running along the homolateral diaphragmatic coupola that drained into superior vena cava and malposition of the mesenterial vessels without any intestinal malrotation. Anomalies of the fetal cardinals and umbilical veins are the result of early abnormal venous system development in the embryo, the etiology of which is unknown. Usually, abnormal systemic venous return only affects one vessel. The presence of a double anomaly as described in this case is exceptional.A persistent right umbilical vein is reported in 0.2 to 0.4% of antenatal screening. The variant with intrahepatic transition is the most frequent and isolated form. The exceptional, form with hepatic bypass, is commonly part of a polymalformative syndrome. Agenesis of the inferior vena cava (IVC) with azygos continuity is a rare anomaly (0,2-3% of antenatal screening). Its significance relates to its frequent association with complex congenital heart diseases. The prognosis of anomalies of cardinals and umbilical veins depends on the presence of associated cardiac and extra cardiac malformations that might impact prenatal counseling and parental decision to continue or interrupt pregnancy. Careful repeated pre and post-natal evaluation is necessary to exclude polymalformative syndrome despite of normal molecular genetic examination.

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The fetal venous system, Part II: ultrasound evaluation of the fetus with congenital venous system malformation or developing circulatory compromise

Cited by 108 publications

References 135 publications

The Anomalies of Systemic Venous Connections in Children with Congenital Heart Disease

The Anomalies of Systemic Venous Connections in Children with Congenital Heart Disease

Diagnóstico prenatal y evolución en fetos con agenesia de ductus venoso

Persistent Right-Sided Umbilical Vein and Azygos Continuity associated with Ophthalmological and Pulmonary Malformations. Prenatal Diagnosis of an Exceptional Case

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