Prenatal Detection and Postnatal Outcome of Persistent Left Superior Vena Cava and Agenesis of Ductus Venosus Associated with Postnatal Bovine Aortic Arch.

BACKGROUND: Isolated agenesis of ductus venosus (ADV) is usually a benign condition, but it may be associated with cardiovascular defects, hydrops, growth restriction, and chromosomal abnormalities. Additionally, persistent left superior vena cava (PLSVC) and bovine aortic arch are relatively common fetal anomalies. To the author's knowledge, this is the first report of prenatal detection of DV agenesis and PLSVC associated with the postnatal bovine aortic arch with a hypoplastic transverse aortic arch. CASE: A 25-year-old, G2P1 woman was referred to our department at 31 weeks due to fetal growth restriction and short femur. On fetal echocardiography, DV could not be viewed via two-dimensional (2D) and Doppler ultrasound (US) imaging; there was also evidence of the co-occurrence of PLSVC and an aortic arch anomaly. We revealed the intrahepatic continuation of the umbilical vein. A weekly follow-up program was scheduled for the patient and the rest of the pregnancy was uneventful. Postnatal, thorax computer tomography and transthoracic echocardiography (TTE) demonstrated PLSVC and bovine aortic arch associated with hypoplastic transverse aortic arch. Routine echocardiographic examinations revealed that the blood flow of the aortic arch had increased gradually, and the male infant's aortic arch had significantly widened and reached the normal range until the baby was discharged from the hospital. CONCLUSION: DV agenesis and PLSVC are usually benign conditions but underlying serious heart diseases may accompany them. Therefore, in situations like ours, a prenatal aortic arch evaluation is of capital importance. Postnatal hemodynamic changes should be taken into consideration in the management of these cases. This is the first example in the literature that these abnormalities co-existed in one case.

Ultrasonographic classification of 26 cases of fetal umbilical-portal-systemic venous shunts and the correlations with fetal chromosomal abnormalities.

OBJECTIVE: To investigate the ultrasonographic classification of fetal umbilical-portal-systemic venous shunts (UPSVS) and the correlations with fetal chromosomal abnormalities. METHODS: We retrospectively analyzed the ultrasound characteristics and the corresponding chromosomal abnormalities of 26 cases of fetal UPSVS prenatally diagnosed. RESULTS: A total of 26 fetuses diagnosed as UPSVS were included, including four cases of type I UPSVS, ten of type II, three of type IIIA, and nine of type IIIB. Four cases of type I were all complicated by fetal heart enlargement and heart insufficiency, of which one case had multiple malformations, and all four cases terminated pregnancies. Six of ten cases of type II terminated pregnancies, including four of Down's syndrome, one of twin reversed arterial perfusion sequence, one of fetal edema but with normal copy number variation (CNV) by chorionic villus sampling. The other four of ten cases were isolated type II with normal chromosomes, which were delivered at full term and were normal in growth and development when followed up 34 months after birth. Three cases of type IIIA all terminated pregnancies, of which one had multiple malformations, one had right multicystic dysplastic kidney, and one had fetal heart enlargement and heart failure. Among nine of type IIIB, seven with chromosomal abnormalities and/ or complicated malformations terminated pregnancies, and two with isolated type IIIB and normal chromosomes were delivered at full term, and were normal in growth and development (one was followed up to 33 months after birth and the other 20 months after birth). CONCLUSION: Fetal UPSVS can be clearly diagnosed and typed by prenatal ultrasonography. Fetal prognosis is determined by the types of UPSVS and complicated malformations and/ or chromosomal abnormalities. The probability of fetal chromosomal abnormalities in UPSVS fetuses is related to the ultrasonographic classification.

Large intra-abdominal umbilical vein varix with absent ductus venosus: The undeniable etiology of fetal heart failure despite associated congenital heart disease.

Fetal intra-abdominal umbilical vein varix (IUVV) is one of the rare anomalies of the umbilical vessels that simulate a cystic structure but with a vascular nature. IUVV usually drains into IVC through ductus venosus (DV), with evidence of an increase in the cardiac preload in most cases. In the current report, we present a fetus with congenital heart disease; however, the association of large IUVV with DV agenesis and direct drainage into the heart resulted in a high output fetal heart failure.

Prenatal diagnosis of the persistent right umbilical vein, incidence and clinical significance.

Prenatal diagnosis of persistent right umbilical vein (PRUV) is important due to accompanying malformations. Pregnant women diagnosed with PRUV were analysed retrospectively. Intrahepatic PRUV was seen in 12 of 10.743 foetuses and its incidence was found to be 0.11%. The gestational week at the time of diagnosis was between 20 and 35 weeks. Six of the cases had additional abnormal sonographic findings (50%) and six cases (50%) were isolated. Major congenital malformations were seen in four (33.3%) foetuses, 75% of which were congenital heart disease (CHD). Genitourinary system anomaly accompanied in two cases (16.6%). Invasive diagnostic tests were applied to three pregnant women and the results were reported as normal karyotype. In PRUV cases, a detailed sonographic examination should be performed, especially the cardiovascular system. Although PRUV cases do not appear to be associated with chromosomal abnormalities, invasive diagnostic tests should be recommended in the presence of concomitant anomalies.Impact statementWhat is already known on this subject? The persistent right umbilical vein (PRUV) is a pathological vascular anomaly, in which the left umbilical vein regresses and the right umbilical vein remains open. PRUV can occur in an isolated form that represents its normal variant or be associated with other major or minor anomalies.What do the results of this study add? Additional abnormal sonographic findings were accompanied in 50% of PRUV, major anomaly was detected in 33.3% of them and cardiovascular abnormalities constituted 75% of foetuses with major anomalies.What are the implications of these findings for clinical practice and/or further research? The presence of concomitant anomalies in PRUV cases is not rare and detailed anatomy screening should be done. The most common accompanying abnormality is seen in the cardiovascular system, so foetuses with PRUV should be evaluated by foetal echocardiography.

Prenatal features, associated co-morbidities and clinical course of agenesis of the ductus venosus in the current era.

OBJECTIVES: Agenesis of the ductus venosus (ADV) has been associated with additional anomalies in up to 83% of cases. We sought to investigate characteristics, co-morbidities and outcomes of ADV in the current era. We hypothesized that rates of cardiac and non-cardiac diagnoses and survival would be higher, due to advances in genetic testing, prenatal diagnosis and surveillance. METHODS: A retrospective series of cases diagnosed at our institution from 2007 to 2018 were identified by searching our database. Cardiac and obstetric charts were reviewed for cardiac and extra-cardiac anomalies, genetic results and outcomes. RESULTS: Fourteen cases were diagnosed at a mean gestational age of 23.9 weeks (range 13-33). All had associated genetic, cardiac or extra-cardiac anomalies. Eight (57%) had cardiac anomalies and one other developed cardiomyopathy by 6 months. Extra-cardiac anomalies were present in 93% (13/14) and genetic diagnoses made in 75% (6/8) of those tested. Cardiac output Z-scores were >2 in 60% (6/10) prior to delivery. Two had hydrops, there was one intra-uterine death, 13 live-births and two neonatal deaths. CONCLUSION: Our cohort had more associated diagnoses and a lower mortality than previously reported. In our experience, high output occurs frequently, however with a relatively low risk of hydrops and intrauterine death.

Ultrasonic detection of fetal persistent right umbilical vein and incidence and significance of concomitant anomalies.

BACKGROUND: Persistent right umbilical vein (PRUV) is characterized by atresia of the left umbilical vein while the right umbilical vein remains open. Given the limited sample size of most studies, the incidence of PRUV and the status of concomitant anomalies may not be fully reflected. Thus, we studied the incidence of fetal PRUV and its concomitant anomalies on a larger scale using our hospital database. This study hoped to address the following questions: Does PRUV increase the risk of fetal anomalies? If the PRUV fetus also has a single umbilical artery (SUA), does the risk of fetal anomaly increase further? What is the positive predictive value of PRUV for fetal anomalies? METHODS: This retrospective study analyzed 756 cases of fetal PRUV at our hospital from January 2007 to April 2017. Prenatal ultrasound and color Doppler images were assessed. All PRUV fetuses underwent echocardiography and detailed ultrasound examinations of other systems. Newborn status was obtained via the database or by telephone follow-up. RESULTS: A total of 435,428 pregnant women underwent prenatal ultrasonography at 16-40 weeks, the incidence of fetal PRUV was 0.17%, and 102 fetuses (13.5%) developed other anomalies. Two complicated cases had trisomy 18. PRUV was associated with a higher incidence of fetal anomalies. When fetal anomalies were classified by body systems, PRUV was associated with a higher incidence of cardiovascular, nervous, urinary, skeletal, digestive, and respiratory system anomalies. The positive predictive values of a PRUV for any fetal anomalies and cardiovascular anomalies were 13.5% (95%CI, 11.2-16.2%) and 5.4% (95%CI, 4.0-7.3%), respectively. SUA further increases the risk of PRUV fetuses with other anomalies and cardiovascular anomalies. CONCLUSIONS: Detailed prenatal ultrasonography and echocardiography should be performed in fetuses with PRUV to rule out anomalies in other systems. When the PRUV is combined with SUA, echocardiography is particularly important. Fetuses with complicated PRUV should undergo chromosomal examination. Although isolated fetal PRUV prognosis is good, complicated PRUV prognosis depends on the type and severity of the concomitant anomalies.

Correlations among Congenital Hepatic Shunt, Absent Ductus Venosus, and Umbilical Vein Shunt Revealed by Prenatal Ultrasound.

OBJECTIVES: Congenital disruptions of the hepatic vasculature such as hepatic vascular shunt and absence of ductus venosus (ADV) are rare and often asymptomatic. Moreover, hepatic vasculature abnormality, ADV, and umbilical shunt are easily missed during ultrasound screening due to insufficient scope of examination. In our study, we analyzed the associations among congenital hepatic shunt, ADV, and umbilical vein (UV) shunt by prenatal ultrasound screening. METHODS: This was a retrospective study of 9 cases of congenital hepatic vascular shunt and 14 cases of isolated ADV identified by prenatal ultrasound screening at Chengdu Women and Children's Center Hospital from 2014 to 2018. The review parameters included ultrasound findings of the fetal hepatic vessels, fetal heart, UV, other malformations, complications, and fetal prognosis. RESULTS: The 9 cases of hepatic shunt included 6 cases of isolated portosystemic shunt, 2 mixed cases of vascular shunt with portosystemic shunt, and 1 case of intrahepatic arteriovenous fistula. Among the 8 total cases of portosystemic shunt, 5 were accompanied by ADV (62.5%). Of the 5 cases of congenital hepatic shunt with ADV, 3 were accompanied by umbilical shunt (60%). Among the 9 cases of hepatic vascular shunt, 6 were accompanied by dilated inferior vena cava and cardiomegaly (66.7%). Of the 19 total ADV cases identified, there were 14 cases without hepatic shunt, 5 with hepatic shunt, and 17 with umbilical shunt (89.4%). Among the 14 ADV cases without hepatic shunt, there were 5 cases with congenital heart defect (35.7%) and 4 with cardiomegaly (28.6%). CONCLUSIONS: Portosystemic shunt, ADV, and umbilical shunt are closely associated. Appreciation of these associations can improve prenatal ultrasound screening for a timely prognosis and initiation of appropriate treatment.

Doppler changes in umbilical artery and ductus venosus during fetoscopic prenatal surgical repair of myelomeningocele.

OBJECTIVE: Cardiac dysfunction has been described in 60% of fetuses undergoing open myelomeningocele (MMC) repair. Routine Doppler evaluation of the umbilical artery (UA) and ductus venosus (DV) is challenging during fetal surgery. The aim of this study was to describe Doppler changes in the UA and DV in a cohort of fetuses during fetoscopic MMC repair. METHODS: This was a retrospective study of fetuses that underwent fetoscopic repair of MMC at a single institution between 2014 and 2017. Ultrasound images taken intraoperatively were analyzed to describe the changes in Doppler parameters of the UA and DV during fetoscopic MMC repair. The timing of Doppler changes was matched with the phase of surgery to determine whether a pattern to their appearance could be ascertained. RESULTS: Of the thirty-five cases of fetoscopic MMC repair performed during the study period, Doppler data were available for 15. Of these, UA Doppler data were obtained in 12 cases, and DV Doppler data were obtained in 13 cases. Of the 12 cases for which there were UA data, nine (75%) showed transient absence of end-diastolic flow (EDF), with five (41.7%) of them progressing to reversal of EDF. Of the 13 cases for which there were DV data, nine had elevated pulsatility index; however, none of them had absent or reversed blood flow during atrial contraction. Of the nine cases with absent EDF in the UA, this abnormality first appeared during fetoscopic repair in five (55.6%), after uterus exteriorization but before CO2 insufflation in two (22.2%), after uterus replacement but before skin closure in one (11.1%) and after CO2 insufflation but before the start of the MMC repair in one (11.1%). Doppler abnormalities in four fetuses with reversed EDF and in three with absent EDF resolved during surgery. Follow-up examination 1 day after surgery demonstrated resolution of UA and DV Doppler abnormalities in all fetuses. CONCLUSION: Transient Doppler abnormalities of the UA, without significant changes in the DV, can be seen during fetoscopic MMC repair. The clinical significance of these Doppler findings remains unclear due to their transient and intermittent nature. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Persistent right umbilical vein in isomerism.

OBJECTIVE: Our objective was to evaluate the incidence of a persistent right umbilical vein in isomerism versus situs solitus. METHODS: For this retrospective, observational, nonrandomized report, we identified fetuses with confirmed right umbilical veins from all patients referred for fetal echocardiography in Southern Nevada between January 2006 and January 2019. RESULTS: For the period January 2006 to January 2019, we identified 89 fetuses with situs solitus and a right umbilical vein from 16 320 women undergoing prenatal cardiac evaluation, resulting in a right umbilical vein incidence of 0.5% in situs solitus. For the same period, we identified 36 fetuses with isomerism and confirmatory umbilical vein imaging. Of the 36, 15 (42%) had right umbilical veins. Of the 15, four of 11 (19%) had left isomerism, and 11 of 15 (73%) had right isomerism (P = .006). CONCLUSIONS: A right umbilical vein is rare with situs solitus and common with isomerism. A right umbilical vein is more common in right isomerism than left isomerism and should alert the clinician to check for isomeric situs, especially right isomerism.

Prenatal diagnosis of persistent right umbilical vein - Incidence and clinical impact. A prospective study.

BACKGROUND: Persistent right umbilical vein (PRUV) is usually an isolated finding but it may be accompanied by other fetal malformations. AIMS: We aimed to determine the incidence of prenatally diagnosed PRUV in a referral population, assess the neonatal outcome and discuss the findings together with those from previous publications. MATERIALS AND METHODS: A total of 2360 women with low-risk singleton pregnancies were examined in the second and third trimesters. A transabdominal convex volume transducer was used. B-mode was applied in each patient. Scanning of the venous system included imaging of the target vessels with two-dimensional colour Doppler mapping. The diagnosis of PRUV was made in a transverse section of the fetal abdomen. Three-dimensional ultrasounds were performed as necessary, when anomalous cases were encountered. RESULTS: The incidence of PRUV in our population was 12/2360 = 0.5%, and it was higher than in other retrospective studies. In 75% (n = 9), PRUV was an isolated finding where delivery was uneventful and the postnatal outcome was favourable. In two cases PRUV was accompanied by omphalocele, and in one case by tetralogy of Fallot and single umbilical artery. CONCLUSIONS: PRUV is an uncommon prenatal finding. Screening for this anomaly can be easily performed in all pregnant patients. A diagnosis of PRUV should be followed by a thorough fetal morphology scan in order to exclude any other malformations, especially those of the cardiovascular system.

Fetal intra-abdominal umbilical vein varix: retrospective cohort study and systematic review and meta-analysis.

OBJECTIVE: To investigate the ultrasound characteristics and outcome of pregnancies with fetal intra-abdominal umbilical vein varix (FIUVV). METHODS: Cases of FIUVV managed at our tertiary university hospital over an 8-year period were reviewed. Information retrieved included gestational age and diameter of the umbilical varix at diagnosis, increase in varix diameter, associated ultrasound or chromosomal anomalies and pregnancy outcome. Furthermore, a systematic review and meta-analysis of series of FIUVV in the literature was performed to assess the incidence of chromosomal anomalies, small-for-gestational age infants and intrauterine fetal demise (IUFD), and to pool odds ratio (OR) estimates on the relationship between the incidence of these outcomes and the presence of additional associated ultrasound anomalies. RESULTS: Thirteen cases of FIUVV were included in the cohort study. Additional ultrasound anomalies were found in two (15.4%) of 13 cases. One case of IUFD was observed and no case of chromosomal anomaly or thrombosis of varix was recorded. A total of five studies comprising 254 cases met the inclusion criteria of the systematic review. FIUVV was associated with additional ultrasound anomalies (non-isolated FIUVV) in 19% (95% CI, 10.9-29.1%) of cases. No case of chromosomal abnormality or IUFD was reported in fetuses with isolated FIUVV. In contrast, in the group of non-isolated FIUVV, the incidence of chromosomal anomalies was 19.6% and that of IUFD was 7.3%, with ORs of 14.8 (95% CI, 2.9-73.0) and 8.2 (95% CI, 1.05-63.1), respectively, when compared with the group of isolated FIUVV. CONCLUSION: When isolated, the outcome of cases affected by FIUVV is usually favorable. In about 20% of cases, additional ultrasound anomalies are found, which are associated with an increased risk for chromosomal abnormalities and IUFD. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Three-dimensional assessment of umbilical vein deviation angle for prediction of liver herniation in left-sided congenital diaphragmatic hernia.

OBJECTIVES: To introduce a new sonographic marker of intrathoracic liver herniation in fetuses with left-sided congenital diaphragmatic hernia (CDH). METHODS: In a consecutive series of fetuses with isolated CDH, an ultrasound volume of the fetal abdomen was acquired. On this volume, offline calculation of the angle formed by the midline of the abdomen (joining the center of the vertebral body to the abdominal insertion of the umbilical cord) and a second line joining the center of the vertebral body to the intra-abdominal convexity of the umbilical vein was carried out to give the umbilical vein deviation angle (UVDA). The UVDA was measured in a group of normal fetuses selected as controls. At follow-up, the presence of liver herniation was investigated in all cases of CDH. UVDA values were compared between the CDH group and controls, and between CDH 'liver-up' vs 'liver-down' cases. A receiver-operating characteristics (ROC) curve was constructed to identify a cut-off value of the UVDA with the highest accuracy in predicting liver herniation in the CDH group. RESULTS: Between 2009 and 2015, 22 cases of left-sided CDH were included in the study group, of which nine cases had liver herniation. Eighty-eight normal fetuses were recruited as controls. The UVDA was significantly higher in the cases vs controls (15.25 ± 7.91° vs 7.68 ± 1.55°; P < 0.0001). Moreover, the UVDA was significantly increased in CDH fetuses with liver-up vs liver-down (21.77 ± 8.79° vs 10.75 ± 2.10°; P < 0.0001). On ROC curve analysis the UVDA showed good prediction of liver herniation (area under the ROC curve, 0.94; P < 0.0001) with the best cut-off of 15.2°, yielding a sensitivity of 89% and a specificity of 100% (P < 0.0001). CONCLUSIONS: In fetuses with CDH, umbilical vein bowing may be quantified by measuring the UVDA using three-dimensional ultrasound. This sonographic marker seems to be an accurate predictor of liver herniation in left-sided CDH. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Ductus venosus agenesis and fetal malformations: what can we expect? - a systematic review of the literature.

Background The ductus venosus agenesis (DVA) is a rare condition with a variable prognosis that relies partly on the presence of associated conditions. The purpose of our study was to analyze the literature regarding the post-natal outcome of fetuses with DVA associated with fetal malformations, in order to discuss the best management options for couples. Methods We performed a systematic review of the literature of MEDLINE and SCOPUS electronic databases in a 25-year period from 1992 to September 2017. Methods We found 340 cases of DVA associated with fetal abnormalities. The most common chromosomal abnormalities were: monosomy X (12/48, 25%), trisomy 21 (11/48, 22.9%) and trisomy 18 (6/48, 12.5%). From the 340 cases with DVA, in 31 cases the umbilical venous shunt type was not reported. Of the fetuses, 60.8% (188/309) had an extrahepatic umbilical venous drainage while 39.2% (121/309) presented an intrahepatic connection. The DVA was associated in 71 cases (23.0%) with cardiac abnormalities, in 82 cases (26.5%) with extracardiac abnormalities and in 85 cases (27.5%) with both cardiac and extracardiac abnormalities. Conclusion DVA associated with both cardiac and extracardiac malformations may confer a poorer fetal outcome, a clinically relevant fact that should clarify what can be expected from this entity and help prenatal counseling.

Choriovitelline placenta: prenatal sonographic imaging and clinical characteristics.

OBJECTIVES: To describe the fetal sonographic characteristics, in-utero natural history and postnatal outcome of choriovitelline placenta, in which the fetal umbilical vein is replaced by the extra-embryonic vitelline circulation. METHODS: This was a retrospective study of pregnancies examined during the period 2010-2014. Fetuses which presented with sonographic criteria of a downward caudal course of an enlarged vein from the umbilical annulus to the hepatic hilum were followed prospectively. Two-dimensional and three-dimensional color Doppler with high-definition flow were used in order to investigate the extra- and intrahepatic venous system. Ultrasound images and volumes were stored digitally, clinical data were obtained from patients' medical files and telephone interviews were conducted regarding the course of the pregnancy, perinatal data and developmental milestones. RESULTS: Four cases were identified during the study period. The mean ± SD gestational age at diagnosis was 19.5 ± 4.3 (range, 13-23) weeks. The characteristic downward course of the persistent vitelline vein was associated with aneurysmal dilatation and anomalous anatomical configuration of the intrahepatic venous system. One case ended with antepartum death at 28 weeks. The mean gestational age at delivery was 34.6 (±5.0) weeks and the birth weight corresponded to the 57.2nd (± 16.8 SD) centile. In two cases, a thrombotic mass was detected in the portal venous system after birth. One necessitated antithrombotic treatment for 6 months; in the other case, spontaneous resolution occurred 7 days after birth. In the three surviving infants, the persistent extrahepatic vitelline vein regressed gradually within 6 months after birth. Neurodevelopment was normal at follow-up aged 1 year and 7 months, 3 years and 6 months and 5 years and 5 months. CONCLUSION: The main clinical importance of choriovitelline placentation derives from the possible formation of thrombus in the portal venous system. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.

Echographic features and perinatal outcomes in fetuses with congenital absence of ductus venosus.

INTRODUCTION: The purpose of this study was to describe the echographic features and perinatal outcomes of fetuses with absence of ductus venosus. MATERIAL AND METHODS: Retrospective review of 10 cases with absence of ductus venosus diagnosed by prenatal ultrasonography between January 2014 and February 2016 at a single referral center. Prenatal findings, umbilical shunting type, perinatal outcomes, and autopsy reports were reviewed. RESULTS: A total of 11 491 fetuses underwent a first- and second-trimester screening during the study period. Ten cases of absence of ductus venosus were diagnosed. All of the fetuses presented an extrahepatic shunt: three fetuses from the umbilical vein to the right atrium and the seven remaining fetuses from the umbilical vein to the inferior vena cava. Major structural defects and fetal effusions were detected in six fetuses. There were two cases of chromosomal abnormalities. Five patients underwent legal termination of pregnancy and five decided to carry to term. In two of these, the absence of ductus venosus anomaly was isolated and had a normal outcome. In the remaining three cases, a follow up of the children showed a variety of adverse outcomes. CONCLUSIONS: The absence of ductus venosus is associated with high rates of adverse perinatal outcomes. The prognosis for this group of anomalies depends on the additional findings with targeted ultrasound. This pathology should lead to a detailed anatomical study and affected fetuses should be closely monitored for signs of congestive heart failure.

Prenatal diagnosis of four-vessel umbilical cord with supernumerary vein varix: A case report and literature review.

Four-vessel umbilical cord with two arteries and two veins, and fetal intra-abdominal umbilical vein varix are rare fetal vascular pathologies, associated with congenital anomalies and an adverse perinatal outcome, respectively. We report a case of a healthy male neonate born at a gestational age of 38 weeks, five days, after prenatal sonographic diagnosis of a four-vessel umbilical cord with a supernumerary vein varix. To the best of our knowledge, this is the first case with this combination.

Fetal umbilical-portal-systemic venous shunt: in-utero classification and clinical significance.

OBJECTIVES: To review our experience with fetal umbilical-portal-systemic venous shunts (UPSVS), to devise an in-utero classification system, and to analyze their clinical and prognostic characteristics. METHODS: This was a retrospective review of cases of UPSVS examined at an academic tertiary referral center over the 14-year period from 2001 to 2014 inclusive. The anatomical origin and drainage of the fetal umbilical, portal, ductus venosus and hepatic venous systems, and the integrity of the intrahepatic portal venous system (IHPVS), were assessed using two- and three-dimensional color Doppler sonography. Fetomaternal clinical characteristics and long-term outcome were investigated by means of medical files, imaging documentation and telephone interviews with the mothers. RESULTS: Forty-four cases with UPSVS were reviewed. Prenatal classification was based on the anatomical origin of the shunt (umbilical, portal or ductal), with cases classified into four types: Type I, umbilical-systemic shunt (n = 9 (20.4%)); Type II, ductus venosus-systemic shunt (n = 19 (43.2%)); and Type III, portal-systemic shunt, divided into two subtypes: Type IIIa, intrahepatic portal-systemic shunt (n = 12 (27.2%)) and Type IIIb, extrahepatic portal-systemic shunt (n = 4 (9.1%)). Each type presented particular clinical and prognostic characteristics. The most important favorable prognostic factors were absence of associated major malformation and presence of the IHPVS. Thus, Type IIIa (intrahepatic portal-systemic shunt) had the best outcome. CONCLUSIONS: Fetal UPSVS are anomalies with a broad spectrum of manifestations and prognoses. Accurate mapping of the IHPVS is paramount for optimizing prenatal counseling and postnatal care. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.

Neonatal Outcomes in Fetuses With a Persistent Intrahepatic Right Umbilical Vein.

OBJECTIVES: A fetal persistent intrahepatic right umbilical vein has been linked to anomalies and genetic disorders but can be a normal variant. We conducted a retrospective review to determine other sonographic findings that can stratify fetuses for further evaluation. METHODS: A total of 313 fetuses had a persistent intrahepatic right umbilical vein identified on 17- to 24-week sonography. The outcome was any major congenital anomaly or an adverse neonatal outcome, which was defined as aneuploidy, fetal demise, or neonatal death. RESULTS: A total of 217 patients (69.3%) had a normal neonatal outcome. Sixty-nine patients (22.0%) were lost to follow-up. Five fetuses (2.1%) had aneuploidy; 4 of the 5 had additional sonographic findings, and 1 had an isolated persistent intrahepatic right umbilical vein. Twenty-four fetuses had a major anomaly in association with the persistent right umbilical vein; 26 additional fetuses had soft sonographic markers associated with karyotypic abnormalities but were chromosomally normal. Of those with adverse neonatal outcomes, 12 had a congenital heart defect (57%). An additional sonographic finding with a persistent intrahepatic right umbilical vein was predictive of a congenital anomaly or an adverse neonatal outcome (P < .001), with a positive predictive value of 44.0% (95% confidence interval, 30.0%-58.7%). An isolated persistent intrahepatic right umbilical vein had a 0.4% risk for a congenital anomaly or an adverse neonatal outcome. CONCLUSIONS: A persistent intrahepatic right umbilical vein should prompt an extended anatomic survey and a fetal cardiac evaluation. If the survey and cardiac anatomy are reassuring, no further follow-up is needed. If additional findings are identified, genetic counseling and invasive testing should be considered.

Intrahepatic Persistent Right Umbilical Vein and Associated Outcomes: A Systematic Review of the Literature.

The aim of this study was to provide a comprehensive review of the current data surrounding an intrahepatic persistent right umbilical vein in the fetus, including associated anomalies and outcomes, and to assist practitioners in counseling and management of affected pregnancies. We performed a MEDLINE, Embase, Cochrane Central Register of Controlled Trials, and Northern Light database search for articles reporting outcomes on prenatally diagnosed cases of a persistent right umbilical vein. Each article was independently reviewed for eligibility by the investigators. Thereafter, the data were extracted and validated independently by 3 investigators. A total of 322 articles were retrieved, and 16 were included in this systematic review. The overall prevalence of an intrahepatic persistent right umbilical vein was found to be 212 per 166,548 (0.13%). Of the 240 cases of an intrahepatic persistent right umbilical vein identified, 183 (76.3%) were isolated. The remaining cases had a coexisting abnormality, including 19 (7.9%) cardiac, 9 (3.8%) central nervous system, 15 (6.3%) genitourinary, 3 (1.3%) genetic, and 17 (7%) placental/cord (predominantly a single umbilical artery). In summary, a persistent right umbilical vein is commonly an isolated finding but may be associated with a coexisting cardiac defect in 8% of cases. Therefore, consideration should be given to fetal echocardiography in cases of a persistent right umbilical vein.

A retrospective single centre review of the incidence and prognostic significance of persistent foetal right umbilical vein.

The objective of our study was to appraise the incidence and significance of persistent right umbilical vein (PRUV), the most common foetal venous aberration. Based on a south Indian antenatal cohort, we identified 23 cases of PRUV amongst 20,452 foetuses of consecutive pregnancies, from 2009 to 2014, yielding an incidence of 1 in 889 total births (0.11%). The median maternal age was 24 (IQR, 22-26) years, and median gestational age at diagnosis was 23 (IQR, 22-24) weeks. Intrahepatic drainage of PRUV was seen in 91.3% cases. In three cases (13%), ductus venosus was absent. In 52.2% of the cases, additional major abnormalities were observed - predominantly cardiovascular (39.1%). The common minor marker was single umbilical artery (13%). The karyotype was found to be normal in six cases (26%) which underwent invasive testing. When associated anomalies were inconsequential or absent, the postnatal outcome was good, which reflected in 60.9% of our cases.