Umbilical-portal-systemic venous shunt and intrauterine growth restriction: an inquiry from a prospective study.

BACKGROUND: The investigation of the fetal umbilical-portal venous system is based on the premise that congenital anomalies of this system may be related to adverse perinatal outcomes. Several small retrospective studies have reported an association between umbilical-portal-systemic venous shunts and intrauterine growth restriction. However, the prevalence of portosystemic shunts in the fetal growth restricted population is yet to be determined. OBJECTIVE: The aims of this study were (1) to determine the prevalence of fetal umbilical-portal-systemic venous shunts in pregnancies complicated by intrauterine growth restriction and (2) to compare the perinatal and neonatal outcomes of pregnancies with intrauterine growth restriction with and without umbilical-portal-systemic venous shunts. STUDY DESIGN: This was a prospective, cross-sectional study of pregnancies diagnosed with intrauterine growth restriction, as defined by the Society for Maternal-Fetal Medicine intrauterine growth restriction guidelines. All participants underwent a detailed anomaly scan, supplemented with a targeted scan of the fetal portal system. Venous shunts were diagnosed using color Doppler mode. The perinatal outcomes of pregnancies with intrauterine growth restriction with and without umbilical-portal-systemic venous shunts were compared. RESULTS: A total of 150 cases with intrauterine growth restriction were recruited. The prevalence of umbilical-portal-systemic venous shunts in our cohort was 9.3% (n=14). When compared with the control group (intrauterine growth restriction without umbilical-portal-systemic venous shunts, n=136), the study group had a significantly lower mean gestational age at the time of intrauterine growth restriction diagnosis (29.7±5.6 vs 32.47±4.6 weeks of gestation; P=.036) and an earlier gestational age at delivery (33.50±6.0 vs 36.13±2.8; P=.005). The study group had a higher rate of fetal death (21.4% vs 0.7%; P<.001) and, accordingly, a lower rate of live births (71.4% vs 95.6%; P=.001). Additional associated fetal vascular anomalies were significantly more prevalent in the study group than in the control group (35.7% vs 4.4%; P≤.001). The rate of other associated anomalies was similar. The study group had a significantly lower rate of abnormal uterine artery Doppler indices (0% vs 40.4%; P=.011) and a higher rate of abnormal ductus venosus Doppler indices (64.3% vs 23%; P=.001). There were no cases of hypertensive disorders of pregnancy in the study group, whereas the control group had an incidence of 12.5% (P=.16). Other perinatal and neonatal outcomes were comparable. CONCLUSION: Umbilical-portal-systemic venous shunt is a relatively common finding among fetuses with growth restriction. When compared with pregnancies with intrauterine growth restriction with a normal portal system, these pregnancies complicated by intrauterine growth restriction and an umbilical-portal-systemic venous shunt are associated with a different Doppler flow pattern, an increased risk for fetal death, earlier presentation of intrauterine growth restriction, a lower gestational age at delivery, additional congenital vascular anomalies, and a lower rate of pregnancy-induced hypertensive disorders. Meticulous sonographic evaluation of the portal system should be considered in the prenatal workup of intrauterine growth restriction, as umbilical-portal-systemic venous shunts may affect perinatal outcomes.

Absence of Ductus Venosus: A Comparison of 2 Distinctive Fetal Autopsy Cases and Embryologic Perspectives.

In fetal circulation, oxygenated blood from the placenta flows through the umbilical vein into the ductus venosus (DV), then enters the inferior vena cava, and subsequently reaches the right atrium of the heart. The DV serves as a shunt, allowing this oxygen-rich blood to bypass the liver. The absence of the DV (ADV), also known as agenesis of the DV, is a rare congenital anomaly. Without a DV, blood from the umbilical vein must follow alternative routes to the heart. In ADV cases, blood from the umbilical vein must follow 1 of 2 primary drainage patterns: either an extrahepatic shunt or an intrahepatic shunt. This report details the antenatal ultrasound and postmortem findings of 2 fetuses diagnosed with ADV by prenatal imaging studies. The first case involved a fetus with a persistent right umbilical vein connected directly to the suprahepatic IVC, accompanied by early obliteration of the left umbilical vein and true agenesis of the DV. This fetus also had additional congenital anomalies. In contrast, the second case involved a fetus with a normal left umbilical vein that entered the liver. However, despite an ultrasound diagnosis of "absence" of the DV, a DV was present, though markedly hypoplastic and probably minimally functional or non-functional. In this case, blood from the umbilical vein likely followed an alternate intrahepatic route through the portal and hepatic veins, before reaching the heart (intrahepatic shunt). These contrasting cases emphasize the heterogeneity of vascular anomalies and embryologic origins captured by the term "ADV." Additionally, the terminology of "absence" or "agenesis" may be misleading in some purported ADV cases. Specifically, in the second case, the DV was not absent; it was markedly hypoplastic instead. This also appears to be the first reported case of a hypoplastic DV in a fetus. Both cases underscore the importance of effective collaboration and clear communication between maternal-fetal medicine specialists and pathologists.

Agenesis of the Ductus Venosus and Its Association With Genetic Abnormalities.

OBJECTIVE: To investigate the association of agenesis of the ductus venosus (ADV) with genetic abnormalities using genetic studies-Chromosomal Microarray Analysis (CMA) and Exome Sequencing (ES). DESIGN: Retrospective study of all fetuses diagnosed with ADV between January 2013 and December 2022 in a tertiary center. RESULTS: ADV was diagnosed in 33 fetuses. The diagnosis was made at a mean gestational age of 21.2 ± 8.4 weeks. Conventional karyotype was applied in a single fetus (3.0%), CMA was applied in 21 fetuses (66.7%), and five fetuses (22.8%) were additionally tested with ES. ADV was isolated in eight fetuses (24%), whereas in 25 (76%) it was associated with abnormal ultrasound findings, including increased nuchal translucency (NT), intrauterine growth restriction (IUGR) and variable structural malformations, mostly cardiac (42%) followed by central nervous system (CNS) and skeletal malformations (24%). Genetic abnormalities were found in six fetuses out of 22 investigated (27%), of which 3 were detected by ES, 3 by CMA and 1 by conventional karyotype. A higher incidence of genetic aberrations was evident among ADVs associated with abnormal ultrasound findings. Genetic abnormalities were indicative of Prader Willi/Angelman syndrome, Noonan syndrome, CASK related disorder, 16q24.3 microdeletion syndrome and Trisomy 21. CONCLUSION: ADV associated with abnormal ultrasound findings is commonly correlated with genetic abnormalities and consequently unfavorable pregnancy outcomes. Our study emphasizes the value of genetic studies chiefly among cases associated with abnormal ultrasound findings, enabling early diagnosis of fetal pathologies associated with ADV, and providing better parental counseling.

Management of fetuses with apparent normal growth and abnormal cerebroplacental ratio: A risk-based approach near term.

INTRODUCTION: Cerebroplacental ratio (CPR) has been shown to be an independent predictor of adverse perinatal outcome at term and a marker of failure to reach the growth potential (FRGP) regardless of fetal size, being abnormal in compromised fetuses with birthweight above the 10th centile. The main aim of this study was to propose a risk-based approach for the management of pregnancies with normal estimated fetal weight (EFW) and abnormal CPR near term. MATERIAL AND METHODS: This was a retrospective study of 943 pregnancies, that underwent an ultrasound evaluation of EFW and CPR at or beyond 34 weeks. CPR values were converted into multiples of the median (MoM) and EFW into centiles according to local references. Pregnancies were then divided into four groups: normal fetuses (defined as EFW ≥10th centile and CPR ≥0.6765 MoM), small for gestational age (EFW <10th centile and CPR ≥0.6765 MoM), fetal growth restriction (EFW <10th centile and CPR <0.6765 MoM), and fetuses with apparent normal growth (EFW ≥10th centile) and abnormal CPR (<0.6765 MoM), that present FRGP. Intrapartum fetal compromise (IFC) was defined as an abnormal intrapartum cardiotocogram or pH requiring cesarean delivery. Risk comparisons were performed among the four groups, based on the different frequencies of IFC. The risks of IFC were subsequently extrapolated into a gestational age scale, defining the optimal gestation to plan the birth for each of the four groups. RESULTS: Fetal growth restriction was the group with the highest frequency of IFC followed by FRGP, small for gestational age, and normal groups. The "a priori" risks of the fetal growth restriction and normal groups were used to determine the limits of two scales. One defining the IFC risk and the other defining the appropriate gestational age for delivery. Extrapolation of the risk between both scales placed the optimal gestational age for delivery at 39 weeks of gestation in the case of FRGP and at 40 weeks in the case of small for gestational age. CONCLUSIONS: Fetuses near term may be evaluated according to the CPR and EFW defining four groups that present a progressive risk of IFC. Fetuses in pregnancies complicated by FRGP are likely to benefit from being delivered at 39 weeks of gestation.

Agenesis of the ductus venosus and fetal growth restriction: Is there a relation? A tertiary care center experience and systematic review of the literature.

An update on the antenatal diagnosis of agenesis of ductus venosus (ADV) by differentiating the various possible types of shunts, focusing on the associated fetal anomalies, and predicting neonatal outcomes. This study reviewed the experience of two tertiary referral centers and literature. An unfavorable outcome was detected in preterm fetuses (p = 0.017), fetuses with a genetic anomaly (p = 0.046) or other associated malformations (p < 0.001). 71% of ADVs with other anomalies had an extrahepatic ADV (p = 0.002). 76% of fetuses with Fetal Growth Restriction (FGR) had an extrahepatic ADV (p = 0.025). ADV may negatively influence fetal growth in cases with extrahepatic vein drainage.

Comparison of ductus venosus Doppler and cerebroplacental ratio for the prediction of adverse perinatal outcome in high-risk pregnancies before and after 34 weeks.

INTRODUCTION: The objective of the study was to compare the accuracy of the ductus venosus pulsatility index (DV PI) with that of the cerebroplacental ratio (CPR) for the prediction of adverse perinatal outcome at two gestational ages: <34 and ≥34 weeks' gestation. MATERIAL AND METHODS: This was a retrospective study of 169 high-risk pregnancies (72 < 34 and 97 ≥ 34 weeks) that underwent an ultrasound examination of CPR, DV Doppler and estimated fetal weight at 22-40 weeks. The CPR and DV PI were converted into multiples of the median, and the estimated fetal weight into centiles according to local references. Adverse perinatal outcome was defined as a composite of abnormal cardiotocogram, intrapartum pH requiring cesarean delivery, 5' Apgar score <7, neonatal pH <7.10 and admission to neonatal intensive care unit. Values were plotted according to the interval to labor to evaluate progression of abnormal Doppler values, and their accuracy was evaluated at both gestational periods, alone and combined with clinical data, by means of univariable and multivariable models, using the Akaike information criteria (AIC) and the area under the curve (AUC). RESULTS: Prior to 34 weeks' gestation, DV PI was the latest parameter to become abnormal. However, it was a poor predictor of adverse perinatal outcome (AUC 0.56, 95% CI: 0.40-0.71, AIC 76.2, p > 0.05), and did not improve the predictive accuracy of CPR for adverse perinatal outcome (AUC 0.88, 95% CI: 0.79-0.97, AIC 52.9, p < 0.0001). After 34 weeks' gestation, the chronology of the DV PI and CPR anomalies overlapped, but again DV PI was a poor predictor for adverse perinatal outcome (AUC 0.62, 95% CI: 0.49-0.74, AIC 120.6, p > 0.05), that did not improve the CPR ability to predict adverse perinatal outcome (AUC 0.80, 95% CI: 0.67-0.92, AIC 106.8, p < 0.0001). The predictive accuracy of CPR prior to 34 weeks persisted when the gestational age at delivery was included in the model (AUC 0.91, 95% CI: 0.81-1.00, AIC 46.3, p < 0.0001, vs AUC 0.86, 95% CI: 0.72-1, AIC 56.1, p < 0.0001), and therefore was not determined by prematurity. CONCLUSIONS: CPR predicts adverse perinatal outcome better than DV PI, regardless of gestational age. Larger prospective studies are needed to delineate the role of ultrasound tools of fetal wellbeing assessment in predicting and preventing adverse perinatal outcome.

Velamentous cord insertion in monochorionic twin pregnancies: a step forward in screening for twin to twin transfusion syndrome and birthweight discordance?

OBJECTIVES: Two major complications of monochorionic diamniotic (MCDA) twin pregnancies are twin to twin transfusion syndrome (TTTS) and birthweight discordance. The current screening ultrasound test for these pathologies combines the detection of nuchal translucency discrepancy and abnormal ductus venosus in at least one twin, in the first trimester. We aim to determine whether combining the presence of velamentous cord insertion in at least one twin increases screening efficiency. METHODS: This was a retrospective cohort with a sample of 136 MCDA twin pregnancies followed at Centro Hospitalar Universitário São João, during a 16-year period. RESULTS: The combination of abnormal ductus venosus in at least one twin and nuchal translucency discrepancy is associated with the development of TTTS with an OR of 10.455, but not with birthweight discordance. The combination of these first trimester markers with velamentous cord insertion is not associated with the development of either outcome. CONCLUSIONS: The presence of velamentous cord insertion in MCDA pregnancies is not associated to TTTS development. Therefore, the addition of this marker to the first trimester screening would not effectively predict the development of birthweight discordance or TTTS. However, a positive currently used screening test increases the risk of developing TTTS by about ten times.

The diagnostic performance of the ductus venosus for the detection of cardiac defects in the first trimester: a systematic review and diagnostic test accuracy meta-analysis.

PURPOSE: Abnormal flow in the ductus venosus (DV) has been reported to be associated with adverse perinatal outcome, chromosomal abnormalities, and congenital heart defects (CHD). Aneuploid fetuses have increased risk of CHD, but there are discrepancies on the performance of this markers in euploid fetuses. The aim of this meta-analysis was to establish the predictive accuracy of DV for CHD. METHODS: MEDLINE, EMBASE, and CINAHL were searched from inception to February 2022. No language or geographical restrictions were applied. Inclusion criteria regarded observational and randomized studies concerning first-trimester DV flow as CHD marker. Random effect meta-analyses to calculate risk ratio (RR) with 95% confidence interval (CI), hierarchical summary receiver-operating characteristics (HSROC), and bivariate models to evaluate diagnostic accuracy were used. Primary outcome was the diagnostic performance of DV in detecting prenatal CHD by means of area under the curve (AUROC). Subgroup analysis for euploid, high-risk, and normal NT fetuses was performed. Quality assessment of included papers was performed using QUADAS-2. RESULTS: Twenty two studies, with a total of 204.829 fetuses undergoing first trimester scan with DV Doppler evaluation, fulfilled the inclusion criteria for this systematic review. Overall, abnormal DV flow at the time of first trimester screening was associated to an increased risk of CHD (RR 6.9, 95% CI 3.7-12.6; I2 = 95.2%) as well in unselected (RR: 6.4, 95% CI 2.5-16.4; I2 = 93.3%) and in euploid (RR: 6.45, 95% CI 3.3-12.6; I2 = 95.8%) fetuses. The overall diagnostic accuracy of abnormal DV in detecting CHD was good in euploid fetuses with an AUROC of 0.81 (95% CI 0.78-0.84), but it was poor in the high-risk group with an AUROC of 0.66 (95% CI 0.62-0.70) and in the unselected population with an AUROC of 0.44 (95% CI 0.40-0.49). CONCLUSIONS: Abnormal DV in the first trimester increases the risk of CHD with a moderate sensitivity for euploid fetuses. In combination with other markers (NT, TV regurgitation) could be helpful to identify fetuses otherwise considered to be at low risk for CHD. In addition to the improvement of the fetal heart examination in the first trimester, this strategy can increase the detection of major CHD at earlier stage of pregnancy.

Palliative stenting of the venous duct in a premature neonate with obstructed infradiaphragmatic total anomalous pulmonary venous connection.

In infracardiac, infradiaphragmatic total anomalous pulmonary venous connection, all four pulmonary veins connect to a descending vertical vein that usually drains to the portal vein or one of its tributaries. Obstruction is common, and definitive treatment is surgical repair. We present a case of late-diagnosed infradiaphragmatic total anomalous pulmonary venous connection in a premature neonate who was too high risk for surgery and underwent palliative stenting of the venous duct. We demonstrate the feasibility of a transhepatic approach when umbilical access is no longer available.

Shunt rate of ductus arteriosus and ductus venosus in middle and late fetuses and their application value in the evaluation of fetal growth restriction.

Objective: To explore the Shunt rate of ductus arteriosus (DA) and ductus venosus (DV) in middle and late fetuses and their application value in the evaluation of fetal growth restriction (FGR). Methods: In this retrospective observational study, we reviewed the clinical data of the patients who admitted to the Second Affiliated Hospital of Wenzhou Medical University from September 10, 2017 to November 27, 2018, and finally included 44 normal women at 28-31 weeks of pregnancy (Normal group) and 15 pregnant women with fetal growth restriction (FGR) within 28-31 weeks of gestation (FGR group). We measured blood flows of the DA (QDA), pulmonary artery (QPA), DV (QDV), and umbilical vein (QUV) and the shunt rates of the DA and DV (QDA/QPA and QDV/QUV, respectively) in all fetuses. We compared the mean variables between groups using the Normal group means as the normal reference values for analysis. Results: DA shunt rate was linearly and positively correlated with gestational age (Y=1.455X+2.787; r=0.767, P<0.01), while the DV shunt rate was linearly and negatively correlated with gestational age (Y=-2.791X+126.885; r=0.761, P<0.01). The DA shunt rates (QDA/QPA) of fetuses in the normal were higher than those in the FGR groups, but the differences between the two groups were not statistically significant (P > 0.05). The DV shunt rates (QDV/QUV) of fetuses in the normal were significantly lower than those in the FGR groups (P < 0.05). The DV shunt rates in the FGR group were significantly higher than those in the normal group with differences being statistically significant at 30-30+6 and 31-31+6 gestational weeks (P < 0.05) The receiver operating characteristic curve (ROC curve) showed that the higher the shunt rate, the worse the birth outcome of a fetus with FGR. Conclusions: The DV shunt rate in middle- and late-stage fetuses can predict the fetal birth outcome, and the higher the shunt ratio, the worse the birth outcome of FGR fetuses.

Reference values for ductus venosus Doppler velocity indices between 11 and 13+6 weeks of gestation: A single-center prospective study in Iran.

Background: This study aimed to investigate reference Doppler velocimetry indices (DVIs) of the fetal ductus venosus (DV) during 11-13 + 6 gestational weeks. Materials and Methods: In a prospective observation over referrals to a single tertiary care center in a 2-year interval, normal singleton pregnancies with fetal crown-rump lengths (CRLs) of 43-80 mm were examined by a single experienced sonographer for their DV pulsatility index (DVPI), DV resistance index (DVRI), and S-wave maximum velocity/A-wave minimum velocity (S/A ratio). Multinomial and quantile regression functions were used to analyze the effect of gestational age (estimated by CRL) on reference values (5th and 95th percentiles of the distribution in each gestational day/week). P < 0.05 was considered significant. Results: Over a sample of 415 participants with a mean/median gestational age of 12 + 1 weeks, no significant correlations were found between the CRL and DVIs using multinomial regression functions (linear model best fitted for all [DVPI: B coefficient = 0.001, P = 0.235] [DVRI: B coefficient = 0.001, P = 0.287] [DV S/A: B coefficient = 0.010, P = 283]). Quantile regression analyses of DVIs' reference values were nonsignificant across the CRL range except for the DVRI ([5th regression line: coefficient = -0.004, P = 0.018] [95th regression line: coefficient = -0.001, P = 0.030]). Conclusion: Reference values for DVPI, DVRI, and DV S/A ratios were established as 0.80-1.39, 0.62-0.88, and 2.57-6.70, respectively. Future meta-analyses and multicenter studies are required to incorporate DV DVIs into an updated universal version of the practice.

Stenting of ductus venosus as a palliation for portal hypertension because of neonatal cirrhosis.

Portal hypertension because of liver cirrhosis is a significant cause of morbidity and mortality. Treatment options in these patients include liver transplant, symptomatic treatment of oesophageal varices via endoscopic treatment and symptomatic management of ascites. Portosystemic shunt creation can be challenging in newborns and infants. We present a newborn with Trisomy 21, severe portal hypertension secondary to neonatal cirrhosis, oesophageal varices and upper GI bleeding, severe ascites refractory to medical management who underwent ductus venosus stenting as a palliative procedure. He demonstrated remarkable clinical improvement with no subsequent upper GI bleed and resolution of ascites till his last follow-up at 12 months of age. To the best of our knowledge, stenting of the patent ductus venosus to create a portosystemic shunt to relieve portal hypertension has not been reported in English literature.

Clinical Opinion: The diagnosis and management of suspected fetal growth restriction: an evidence-based approach.

This study reviewed the literature about the diagnosis, antepartum surveillance, and time of delivery of fetuses suspected to be small for gestational age or growth restricted. Several guidelines have been issued by major professional organizations, including the International Society of Ultrasound in Obstetrics and Gynecology and the Society for Maternal-Fetal Medicine. The differences in recommendations, in particular about Doppler velocimetry of the ductus venosus and middle cerebral artery, have created confusion among clinicians, and this review has intended to clarify and highlight the available evidence that is pertinent to clinical management. A fetus who is small for gestational age is frequently defined as one with an estimated fetal weight of <10th percentile. This condition has been considered syndromic and has been frequently attributed to fetal growth restriction, a constitutionally small fetus, congenital infections, chromosomal abnormalities, or genetic conditions. Small for gestational age is not synonymous with fetal growth restriction, which is defined by deceleration of fetal growth determined by a change in fetal growth velocity. An abnormal umbilical artery Doppler pulsatility index reflects an increased impedance to flow in the umbilical circulation and is considered to be an indicator of placental disease. The combined finding of an estimated fetal weight of <10th percentile and abnormal umbilical artery Doppler velocimetry has been widely accepted as indicative of fetal growth restriction. Clinical studies have shown that the gestational age at diagnosis can be used to subclassify suspected fetal growth restriction into early and late, depending on whether the condition is diagnosed before or after 32 weeks of gestation. The early type is associated with umbilical artery Doppler abnormalities, whereas the late type is often associated with a low pulsatility index in the middle cerebral artery. A large randomized clinical trial indicated that in the context of early suspected fetal growth restriction, the combination of computerized cardiotocography and fetal ductus venosus Doppler improves outcomes, such that 95% of surviving infants have a normal neurodevelopmental outcome at 2 years of age. A low middle cerebral artery pulsatility index is associated with an adverse perinatal outcome in late fetal growth restriction; however, there is no evidence supporting its use to determine the time of delivery. Nonetheless, an abnormality in middle cerebral artery Doppler could be valuable to increase the surveillance of the fetus at risk. We propose that fetal size, growth rate, uteroplacental Doppler indices, cardiotocography, and maternal conditions (ie, hypertension) according to gestational age are important factors in optimizing the outcome of suspected fetal growth restriction.

Doppler Waveform Analysis of Intertwin Venous Blood Flow in Twin Reversed Arterial Perfusion (TRAP) Sequence with a Rudimentary Heart.

Twin reversed arterial perfusion (TRAP) sequence is a rare anomaly in 1% of monochorionic twin pregnancies. Few TRAP sequence cases have a rudimentary heart with cardiac motion in the acardiac twins. Herein, we investigated the venous Doppler waveform in two cases of TRAP sequence with a rudimentary heart with cardiac motion in the acardiac twin. Although both cases had veno-venous anastomoses, the venous Doppler waveforms of the umbilical vein differed. A comparison of the characteristics of the cases indicated that the existence of a ductus venosus, or greater heart pulse power, in the acardiac twin might impact the venous Doppler waveform in the pump twin.

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.

Physiological fetal vascular shunts and failure to regress: what the radiologist needs to know.

The fetal circulation is characterized by the presence of three physiological vascular shunts - the ductus arteriosus, the foramen ovale and the ductus venosus. Acting in concert, these shunts preferentially stream blood flow in a pattern that maximizes efficiency of blood oxygenation by the maternofetal unit. Shortly following the transition to extrauterine life, a quick and predetermined succession of events results in closure of these embryological structures with consequent establishment of postnatal vascular flow patterns. While this transition is often seamless, the physiological shunts of the fetus occasionally fail to regress. Such failure to regress can occur in isolation or in association with other congenital malformations. This failed regression challenges the circulatory physiology of the neonate and might have implications for the optimum functioning of several organ systems. When symptomatic, these shunts are treated. Interventions, when undertaken, might be medical, endovascular or surgical. The radiologist's role continues to expand in the assessment of these shunts, in providing a roadmap for treatment and in prompt identification of treatment-related complications. This review is to familiarize radiologists with the embryology, pre- and post-treatment imaging appearances, and associated complications of persistent fetal vascular shunts.

Comparison of cardiac morphology and function in small for gestational age fetuses and fetuses with late-onset fetal growth retardation.

OBJECTIVES: To compare cardiac structural and functional findings of fetuses with fetal growth restriction (FGR) and small for gestational age (SGA). METHODS: In this prospective cohort study, patients were classified into three groups using Delphi procedure according to fetal weight, umbilical, uterine artery Doppler and cerebroplacental ratio. Fetal cardiac ultrasonographic morphology and Doppler examination was performed to all pregnant women at 36 weeks of gestation. RESULTS: Seventy three patients were included in the study. There were one (6.7%) patient in the control group, 2 (13.3%) in the SGA group and 12 (80%) in the FGR group who needed neonatal intensive care unit (NICU) and NICU requirement was significantly higher in FGR fetuses (p<0.001). Left spherical index was found to be lower only among FGR fetuses (p=0.046). Left ventricular wall thickness was decreased and the right/left ventricular wall ratio was increased in FGR fetuses (p=0.006, p<0.001). Tricuspid/mitral valve ratio and mitral annular plane systolic excursion value was lower in FGR fetuses (p=0.034, p=0.024 respectively). Also, myocardial performance index was remarkably higher in FGR group (p=0.002). CONCLUSIONS: We detected cardiac morphological changes in cases of both SGA and FGR-more pronounced in the FGR cases. Findings related to morphological changes on the left side in FGR cases were considered secondary to volume increase in FGR cases as an indicator of a brain-protective effect. In the FGR group, both systolic and diastolic dysfunctions were detected in the left heart.

Stepwise Treatment for Heterotaxy Syndrome and Functional Single Ventricle Complicated by Infra-Cardiac Total Anomalous Pulmonary Venous Connection with Ductus Venosus Stent Placement and Subsequent Occlusion.

Even today, when the surgical outcome of congenital heart disease in the neonatal period has improved, the prognosis for heterotaxy syndrome and functional single ventricle complicated with total anomalous pulmonary venous connection (TAPVC), especially the infra-cardiac type, is catastrophic. We describe a strategy that combines percutaneous ductus venosus (DV) stent placement and occlusion after TAPVC repair to ensure survival from initial surgery to bidirectional cavopulmonary shunt (BCPS) procedure and facilitate subsequent treatment. Three consecutive patients with heterotaxy syndrome and functional single ventricle complicated by infra-cardiac TAPVC treated with our own strategy were retrospectively studied. In two infants, DV stent placement was performed on the day of birth. In one case at 11 days of age. The risk of pulmonary vein obstruction was reduced, and on-pump surgery, including TAPVC repair, was performed on a standby basis. Since the rapid increase in hepatic enzymes occurred on postoperative day 0 to 1 in all cases, percutaneous stent occlusion was performed until postoperative day 3. The procedure improved liver function. One patient died due to severe atrioventricular valve regurgitation, one case underwent BCPS, and one patient was waiting to undergo. DV stent placement can avoid TAPVC repair in the early neonatal period. After TAPVC repair, the portosystemic shunt remained, resulting in hepatic dysfunction, but this could be improved by stent and vertical vein occlusion. A series of stepwise treatments can be useful to help such critically ill infants survive the high-risk neonatal period and achieve good BCPS circulation.

An unusual umbilical venous connection to a left posterior intercostal vein.

A foetal echocardiogram, in a 27-week foetus referred for cardiomegaly, demonstrated dextrocardia, absence of the ductus venosus, and an unrestricted unusual umbilical venous drainage to a left posterior intercostal vein, which continued to left hemiazygos vein and drained into the coronary sinus. Progressive cardiomegaly led to early delivery. To the best of our knowledge, no case with similar umbilical venous drainage has been previously reported.

Imaging of fetal precordial venous system by four-dimensional ultrasound with spatiotemporal image correlation technology.

Despite the use of two- and three- dimensional color Doppler ultrasonography, the prenatal sonographic evaluation of precordial venous system remains difficult. Spatiotemporal image correlation (STIC) technology has been well described for the assessment of the fetal heart, but not of precordial venous system. As demonstrated in this report, it is feasible to use four-dimensional ultrasound with STIC rendered volume in glass-body mode and tomographic ultrasound imaging to image this system including the connection and drainage of the ductus venosus, hepatic veins and inferior vena cava to the fetal heart. This novel approach can increase the understanding of this venous system.