Complex Arterio-Portal-Venous Malformation of the Liver.

A neonatal female patient exhibited a congenital intricate vascular malformation affecting the liver, encompassing anomalies in the arterial, venous, and portal venous systems and notably including an aneurysm within the portal vein. The management strategy involved a staged endovascular approach, initially using retrograde embolization via the venous outflow tract. Subsequently, transarterial embolization was performed to address complications associated with pulmonary and portal hypertension.

Repair of infracardiac total anomalous pulmonary venous connection in a 902-gram infant with asplenia syndrome after failed umbilical venous catheter intervention.

Ductus venosus stenting via a transumbilical approach for pulmonary venous obstruction in infracardiac total anomalous pulmonary venous connection has been described. In a 902-gram infant who was diagnosed with asplenia syndrome and infracardiac total anomalous pulmonary venous connection, ductus venosus stenting was attempted by a transumbilical approach. However, ductus venosus stenting was discontinued due to bleeding from the portal vein. The bleeding subsided in time spontaneously, and total anomalous pulmonary venous connection repair with pulmonary artery banding was performed on 21 days after birth. To our knowledge, this is the first report that describes total anomalous pulmonary venous connection repair in a neonate under 1000 g body weight. Learning objective: Ductus venosus stenting is an effective palliative option, especially in the presence of high surgical risk, such as heterotaxy syndrome and a low birth weight. However, ductus venosus stenting should carefully be evaluated by assessment of anatomical configuration of umbilical vein and ductus venosus. If ductus venosus stenting is anatomically difficult, primary surgical repair may be an option even in an extremely low birth weight infant.

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.

Regional differences in the umbilical vein and ductus venosus at different stages of normal human development.

During the fetal period, oxygenated blood from the placenta flows through the umbilical vein (UV), portal sinus, ductus venosus (DV), and inferior vena cava (IVC) to the heart. This venous route varies regionally in many aspects. Herein, we sought to characterize the venous route's morphological features and regional differences during embryonic and early-fetal periods. Twenty-nine specimens were selected for high-resolution digitized imaging; 18 embryos were chosen for histological analysis. The venous route showed a primitive, large, S-shaped curved morphology with regional narrowing and dilation at Carnegie stage (CS) 15. Regional differences in vessel-wall differentiation became apparent from approximately CS20. The vessel wall was poorly developed in most DV parts; local vessel-wall thickness at the inlet was first detected at CS20. The lumen of the venous route changed from a nonuniform shape to a relatively round and uniform morphology after CS21. During the early-fetal period, two large bends were observed around the passage of the umbilical ring and at the inlet of the liver. The length ratio of the extrahepatic UV to the total venous route increased. The sectional area gradually increased during embryonic development, whereas differences in sectional area between the DV, UV, and IVC became more pronounced in the early-fetal period. Furthermore, differences in the sectional area between the narrowest part of the DV and other hepatic veins and the transverse sinus became more pronounced. In summary, the present study described morphological, morphometric, and histological changes in the venous route throughout embryonic and early-fetal development, clarifying regional characteristics.

Umbilical Vein Calcification Associated with Double-Lumen Catheter Malpositioning in an Extremely Low-Birth-Weight Infant.

Umbilical venous (UV) catheters (UVCs) are commonly used in severely ill neonates. Complications associated with UVC often result from an inappropriate UVC position. Calcification of the UV, a rare complication, was observed in an extremely low-birth-weight infant born at 23 weeks of gestation. After birth, the infant experienced respiratory and circulatory dysfunction, followed by disseminated intravascular coagulation (DIC). A UVC was inserted, and circulatory agonists and blood transfusions were administered, as well as a calcium gluconate infusion for hypocalcemia and hyperkalemia. Ten days after birth, calcification was detected in the UV, likely due to a tunica intima injury caused by UVC, a hypercoagulable state due to DIC, and a high-dose calcium gluconate infusion. Additionally, proximal port malpositioning of the double-lumen catheter might have contributed to calcification within the UV. To prevent such complications, real-time ultrasound confirmation with agitated saline contrast during UVC placement is recommended; in the absence of the facility or skills for ultrasonography, X-rays should be performed in the lateral and anteroposterior views. Furthermore, when using multi-lumen catheters, physicians should not only verify the tip position but also ensure proper placement of proximal ports and carefully select medications administered through the ports.

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.

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.

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.

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.

Subclassification of fetal growth restriction type IIa vs type IIb applied to twin-twin transfusion syndrome.

BACKGROUND: Subclassification of monochorionic twins with selective fetal growth restriction type II into IIa vs IIb has been proposed because of differing neonatal survival outcomes of the fetus with growth restriction after laser surgery based on preoperative Doppler findings in the middle cerebral artery and ductus venosus. There is substantial clinical overlap between selective fetal growth restriction and twin-twin transfusion syndrome. OBJECTIVE: This study aimed to compare donor twin neonatal survival after laser surgery in cases of twin-twin transfusion syndrome with concomitant donor fetal growth restriction type IIa vs IIb. STUDY DESIGN: This was a retrospective study of monochorionic multifetal pregnancies treated with laser surgery for stage III twin-twin transfusion syndrome and concomitant donor twin fetal growth restriction type II at a referral center from 2006 to 2021. Donor fetal growth restriction type II was defined as having an estimated fetal weight <10th percentile with persistent absent and/or reversed end-diastolic velocity in the umbilical artery. Moreover, patients were subclassified as type IIa (having normal middle cerebral artery peak systolic velocities and ductus venosus Doppler waveforms) vs type IIb (having middle cerebral artery peak systolic velocities ≥1.5 multiples of the median and/or ductus venosus with persistent absent or reversed atrial systolic flow). This study compared 30-day neonatal survival of the donor twin by fetal growth restriction type IIa vs IIb using logistic regression to adjust for relevant preoperative covariates (P<.10 in bivariate analysis). RESULTS: Of 919 patients who underwent laser surgery for twin-twin transfusion syndrome, 262 had sstage III donor or donor and recipient twin-twin transfusion syndrome; of these patients, 189 (20.6%) had concomitant donor fetal growth restriction type II. Moreover, 12 patients met the exclusion criteria, yielding 177 patients (19.3%) who composed the study cohort. Patients were subclassified as donor fetal growth restriction type IIa (146 [82%]) vs type IIb (31 [18%]). Donor neonatal survival for fetal growth restriction type IIa vs IIb was 71.2% vs 41.9% (P=.003). Recipient neonatal survival did not differ between the 2 types (P=1.000). Patients classified with twin-twin transfusion syndrome and concomitant donor fetal growth restriction type IIb were 66% less likely to have neonatal survival of the donor after laser surgery (adjusted odds ratio, 0.34; 95% confidence interval, 0.15-0.80; P=.0127). The logistic regression model was adjusted for gestational age at the procedure, estimated fetal weight percent discordance, and nulliparity. The c-statistic was 0.702. CONCLUSION: For patients with stage III twin-twin transfusion syndrome and concurrent donor fetal growth restriction with persistent absent or reversed end-diastolic velocity in the umbilical artery (ie, fetal growth restriction type II), subclassification into fetal growth restriction type IIb based on elevated middle cerebral artery peak systolic velocity and/or abnormal ductus venosus flow in the donor conveyed poorer prognosis. Although donor neonatal survival after laser surgery was lower for patients with stage III twin-twin transfusion syndrome with donor fetal growth restriction type IIb than patients with stage III twin-twin transfusion syndrome with donor fetal growth restriction with type IIa, laser surgery for fetal growth restriction type IIb in the setting of twin-twin transfusion syndrome (as opposed to pure selective fetal growth restriction type IIb) still allows for the possibility of dual survivorship and should be offered with shared decision-making when counseling patients on management options.

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.

Ductus venosus opens in high-risk pregnancies without signs of increased central venous pressure.

OBJECTIVES: It has been belived that changes in diastolic blood velocities in the fetal ductus venosus were due to increased central venous pressure secondary to increased fetal heart strain during hypoxia or heart failure. There have been recent reports of changes in ductus venosus blood velocity without signs of increased fetal heart strain. The aim of this evaluation was to compare blood velocity in the right hepatic vein as a marker of increased central venous pressure in relationship to changes in ductus venosus blood velocity. MATERIAL AND METHODS: Fifty pregnancies suspected of fetal growth resitriction were evaluated by Doppler ultrasound. Blood velocity was recorded in the right hepatic vein, ducus venosus and in the umbilical vein. Placental blood flow was also recorded in the uterine and umbilical arteries as well as the fetal middle cerebral artery. RESULTS: Increased umbilical artery pulsatility index was recorded in 19 fetuses and 20 has signes of brain sparing according to recordings in the middle cerebral artery. Abnormal blood velocity in the ductus venosus was recorded in 5 fetuses, none of these fetuses had an abnormal pulsatility in the right hepatic vein. CONCLUSIONS: Opening of the ductus venosus is not only related to fetal cardiac strain. This might indicate that the ductus venosus does not primarily open due to increased central venous pressure in moderate fetal hypoxia. Increased fetal cardiac strain might be a late event in the process of chronic fetal hypoxia.

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.

Effective long-term sirolimus treatment in hypoxemia mainly due to intrapulmonary right-to-left shunt in a patient with multiple vascular anomalies.

Pulmonary arteriovenous malformations (PAVMs), particularly where feeding artery/arteries to PAVMs ≥ 3 mm can be treated with embolization. The treatment for hypoxemia resulting from multiple small or diffuse PAVMs remains unclear.We report a girl aged 5 years and 10 months presented with cyanosis and decreased activity after exercise (83-85% of pulse oxygen saturation, SpO2). She had 1 skin lesion on her face and 1 suspected hemangioma on her left upper extremity at birth and that gradually disappeared spontaneously. Physical examination revealed clubbed fingers, and abundant vascular networks on her back. Contrast-enhanced lung CT (slice thickness:1.25 mm) with vascular three-dimensional reconstruction and abdominal CT revealed increased bronchovascular bundles, increased diameter of the pulmonary artery and ascending aorta, and intrahepatic portosystemic venous shunts due to patent ductus venosus. Echocardiography revealed increased diameter of aortic and pulmonary artery. Transthoracic contrast echocardiography was highly positive (bubble appearing in the left ventricle after 5 cardiac cycles). Abdominal doppler ultrasound revealed hepatic-portal venous shunt. Magnetic resonance imaging, artery and vein of the brain revealed multiple malformations of venous sinuses. The patient received sirolimus for 2 years and 4 months. Her condition improved significantly. SpO2 gradually increased to 98%. Her finger clubbing gradually normalized.Our report implicates sirolimus might be a potential treatment option in persistent hypoxemia mainly due to intrapulmonary right-to-left shunt even small multiple or diffusive PAVMs in pediatric patients with multiple cutaneous and visceral vascular anomalies.

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.

Maternal Steroids on Fetal Doppler Indices, in Growth-Restricted Fetuses with Abnormal Umbilical Flow from Pregnancies Complicated with Early-Onset Severe Preeclampsia.

Corticoids are largely used for fetal interest in expected preterm deliveries. This study went further, evaluating the effect of maternal administration of dexamethasone (Dex) on the umbilical artery (UA), middle cerebral artery (MCA), and ductus venous (DV) spectrum, in growth-restricted fetuses, with the absent end-diastolic flow (AEDF) in UA, from singleton early-onset severe preeclamptic pregnancies. Supplementary, the impact on both uterine arteries (UTAs) flow was also evaluated. In 68.7% of cases, the EDF was transiently restored (trAEDF group), in the rest of 31.2% remained persistent absent (prAEDF group). UA-PI significantly decreased in the first day after Dex (day 1/0; p < 0.05), reaching its minimum during day 2 (day 2/1; p > 0.05), revealing a significant recovery to day 4 (day 4/2; p < 0.05), in both groups. The MCA-PI decreased from day 1 until day 3 in both groups, but significantly only in the trAEDF group (p = 0.030 vs. p = 0.227. The DV-PI's decrease (during day 1) and the CPR's increase (between days 0 and 2) were not significant in both groups. UTAs-PIs did not vary. The prAEDF group had a significantly increased rate of antenatal worsening Doppler and a poorer perinatal outcome compared with the trAEDF group. In conclusion, Dex transiently restored the AEDF in UA in the majority of cases, a "positive" effect being a useful marker for better perinatal prognosis. UA-PI significantly decreased in all cases. The improvement in umbilical circulation probably was responsible for the short but not significant DV-PI reduction. MCA-PI decreased only in sensitive cases, probably due to an already cerebral "full" vasodilation in the prAEDF group. Furthermore, the CPR's nonsignificant improvement was the result of a stronger effect of Dex on UA-PI than on MCA-PI. Finally, despite the same etiology, it was only a weak correlation between the severity of the umbilical and uterine abnormal spectrum.

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.

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.

The pre-eclampsia, growth restriction, and ductus venosus doppler (GRADED) study: An observational study of early-onset fetal growth restriction and pre-eclampsia.

OBJECTIVE: To assess the feasibility of using ductus venosus Doppler (DVD) to time delivery in early-onset fetal growth restriction (FGR) and pre-eclampsia in a resource-constrained setting. METHODS: This was a prospective, observational study of pregnancies affected by early-onset FGR and pre-eclampsia. Patient characteristics, risk factors, ultrasound findings, and pregnancy outcomes were entered into a data collection tool. The association of these variables with perinatal and maternal outcomes were determined using binary logistic regression analysis. RESULTS: The study had 61 participants. Most patients were delivered at 29-31+6 weeks of pregnancy (67%). Neonates with an estimated fetal weight on ultrasound of less than 800 g had the highest incidence of perinatal mortality (63%). There was a near six-fold increase in risk of major neonatal morbidity in patients with abnormal DVD (odds ratio 5.9, 95% confidence interval [CI] 1.8-19.0). Absent flow in the DVD a-wave carried a higher risk of perinatal mortality (OR 23.8, 95% CI 1.7-334.8); 22% of patients with an abnormal DVD a-wave experienced placental abruption. CONCLUSION: Having an abnormal DVD in the background of pre-eclampsia was related to increased perinatal morbidity and mortality, with increased risk of placental abruption.