An unusual anomalous intrahepatic connection between the left portal vein and internal mammary veins

Persistent embryonic portal vessels such as the umbilical vein or ductus venosus are occasionally encountered in the adult on ultrasound examination of the liver. These vessels must be identified and distinguished from the normal intrahepatic portal and hepatic veins. Recently, we have found another vein that, like the ductus venosus and persistent umbilical vein, is also a large intrahepatic portal vessel that is not a normal component of the hepatic vasculature. This anomalous vein originated from the left portal vein and traversed the liver parenchyma without branching to reach the anterior-superior liver margin, serving as an intrahepatic portal-systemic shunt by directly connecting the left portal vein and the internal mammary veins.     

Diagnosis of absent ductus venosus in a population referred for fetal echocardiography: association with a persistent portosystemic shunt requiring postnatal device occlusion.

OBJECTIVE: The purpose of this series was to assess the incidence, anatomic variants, and implications of an absent ductus venosus (ADV) in patients referred for fetal echocardiography. METHODS: We searched our fetal cardiology database for diagnoses of ADV from May 2003 to December 2006. RESULTS: During the study period, we performed 1328 fetal echocardiographic examinations in 990 fetuses. We found 6 cases of ADV (6/1000). Indications for fetal echocardiography were cardiomegaly, dilated umbilical or systemic veins, and extracardiac abnormalities. We identified 5 anatomic variants of ADV. In 2 patients, the umbilical vein connected to the systemic venous circulation by way of the portal sinus: via an abnormal venous channel from the portal sinus to the right atrium (case 1) and presumably via hepatic sinusoids to the hepatic veins (case 2). In the remaining 4 patients, the umbilical vein bypassed the portal sinus and the liver and connected to the systemic venous circulation via an abnormal venous channel: from the umbilical vein to the right atrium (case 3), from the umbilical vein to the inferior vena cava (cases 4 and 5), and from the umbilical vein to the right iliac vein (case 6). All patients survived; 2 required cardiovascular intervention. No intervention was required in 3 patients. CONCLUSIONS: An ADV should be ruled out in a fetus with unexplained cardiomegaly or dilatation of the umbilical vein, systemic veins, or portal sinus. To our knowledge, prenatal diagnosis of an ADV with an abnormal communication between the portal sinus and the right atrium has not been reported previously. The portosystemic communication persisted after birth and required device occlusion.     

Prenatal diagnosis of abnormal course of umbilical vein and ductus venosus agenesis: Report of three cases

Ductus venosus connecting the portal and embryonic venous circulation into the inferior vena cava has a crucial role in fetal circulation. The absence of ductus venosus is a rare anomaly, in which the umbilical vein connection to the venous system may be extrahepatic, bypassing the liver or intrahepatic via the portal venous system. We report three cases of ductus venosus agenesis with associated anomalies. In two of them the connection was directly to the right atrium, whereas the umbilical vein drained to the left internal iliac artery in the third case. © 2012 Wiley Periodicals, Inc. J Clin Ultrasound, 2012;     

胎儿脐静脉-门静脉系统正常解剖与异常分流的超声特征分析

目的探讨胎儿脐静脉-门静脉系统的正常超声图像以及异常分流的超声诊断特征。 方法回顾性选取2018年1月至2020年12月在郑州大学第三附属医院行产前超声筛查和诊断的正常胎儿85例,观察脐静脉-门静脉系统的超声图像特征。另收集产前超声诊断为脐静脉-门静脉系统异常分流的26例胎儿的资料,对其产前超声图像及合并异常进行分析。 结果正常胎儿脐静脉-门静脉系统中门静脉主干与门静脉窦连接方式分为3种：“T”形连接60例;“X”形连接11例;“H”形连接14例。脐静脉-门静脉系统异常分流的26例胎儿中,脐静脉-体静脉分流6例,其中脐静脉回流入右心房4例,回流入下腔静脉1例,肝静脉异常分流1例,6例均合并静脉导管缺失,3例合并畸形,3例合并心脏增大;静脉导管-体静脉分流4例,其中静脉导管下腔静脉入口位置下移2例,经冠状静脉窦回流入右心房1例,直接回流至肝静脉1例,2例合并心内、心外多发畸形;肝内门静脉-体静脉分流16例,Ⅰ型1例、Ⅱ型6例、Ⅲ型3例、Ⅳ型6例。其中4例合并胎儿生长受限,2例合并心脏畸形,2例合并单脐动脉。 结论正确识别正常脐静脉-门静脉系统超声图像有助于发现和诊断异常分流;各类型异常分流有不同的超声图像特征;评估肝内门静脉系统发育、心功能及各系统发育可为临床咨询和处理提供信息和依据。     

静脉导管缺如并脐静脉异常连接产前超声心动图表现

目的 观察静脉导管缺如(ADV)并脐静脉肝内、肝外异常连接的产前超声心动图表现。方法 回顾性分析14胎ADV胎儿的超声心动图表现,观察心脏大小、功能变化,并随访临床预后。结果 ADV的检出率为0.12%(14/11 205),14胎中,5胎脐静脉与门静脉左支、1胎与门静脉右支相连;8胎脐静脉未与门静脉相连,而与肝外静脉系统相连,5胎直接连接于右心房,1胎直接连接于下腔静脉,1胎连接于肝右静脉,1胎连接于冠状静脉窦引流入右心房。结论 产前超声心动图能够明确诊断静脉导管缺如,且可以观察脐静脉肝内、肝外异常连接途径和管道内径。     

Prenatal diagnosis of abnormalities of the fetal venous system.

OBJECTIVE: To present our experience in the prenatal diagnosis of anomalies of fetal veins using high-resolution color Doppler ultrasound. DESIGN: An observational study of 16 fetuses with abnormalities of the umbilical, portal, hepatic and caval venous system being diagnosed at the Division of Prenatal Diagnosis and Therapy (Bonn, Germany) over the past 5 years. The abnormality of the venous system, the underlying embryologic disorder and the outcome of the pregnancy are presented and compared with the literature. RESULTS: In group A, eight fetuses had an abnormal course of the umbilical vein with a patent (n = 3) or absent (n = 5) ductus venosus. No portal veins and absent or abnormal hepatic veins were visualized by color Doppler sonography. Six fetuses (75%) did not have an associated malformation and have survived. Two pregnancies with fetal hydrops due to a small heart and to Turner's syndrome were terminated or ended in fetal demise. In group B, seven of eight fetuses with an abnormal caval system had a situs ambiguus or an atrial isomerism. A cardiac defect was detected in six cases (86%). These six pregnancies ended in four terminations of pregnancy and two infant deaths due to the severity of the congenital cardiac defect. One child with a normal heart and a child with an isolated abnormal course of the lower inferior vena cava are developing well. CONCLUSIONS: In a targeted fetal scan the course of the umbilical vein, ductus venosus, the portal and hepatic veins and inferior vena cava should be carefully examined using color Doppler. Any suspicious finding should be followed by a detailed assessment of the specificity of this abnormality taking into consideration the embryologic development of the fetal venous system together with the associated malformations.     

Absence of the ductus venosus: report of 10 new cases and review of the literature.

OBJECTIVE: To assess the clinical significance of the absence of the ductus venosus. DESIGN: A retrospective study with a review of the literature. METHODS: The archives of our ultrasound laboratory and the English literature were searched for cases with a prenatal diagnosis of absence of the fetal ductus venosus. RESULTS: Between 1985 and 2000, 10 fetuses were diagnosed in our center as having absence of the ductus venosus. The review of the literature revealed 23 cases. Three main patterns of abnormal venous circulation were documented: (1) umbilical vein bypassing the liver and connecting directly to the right atrium (46%); (2) umbilical vein bypassing the liver and connecting to the inferior vena cava mostly through one of the iliac veins (25%); (3) umbilical vein connecting to the portal circulation without giving rise to the ductus venosus (21%). Major anomalies, including chromosomal aberrations, were found in 8/33 (24%) cases. Hydrops developed in 11/33 (33%) cases. Twenty fetuses with isolated absence of the ductus venosus were delivered, and 5 (20%) died. The portal vein was found to be absent in half of the infants examined after birth. CONCLUSIONS: Our results and the review of literature suggest that absence of the ductus venosus is associated with a high incidence of fetal anomalies and adverse outcomes, including associated malformations, chromosomal aberrations, in utero heart failure and absence of the portal vein. Heart failure and absence of the portal vein seem particularly frequent when absence of the ductus venosus is associated with a connection of the umbilical vein to either the inferior vena cava or the right atrium.     

Venous Doppler velocimetry in the surveillance of severely compromised fetuses.

OBJECTIVE: To investigate whether venous Doppler velocimetric signs of cardiac decompensation might predict fetal demise in severely compromised fetuses. MATERIAL AND METHODS: This was a prospective study involving 154 growth-restricted fetuses, 37 of which were found to have reversed flow in the umbilical artery (BFC III). Doppler velocimetry of the right hepatic vein and ductus venosus were investigated serially and the presence of umbilical venous pulsations also registered. Only the final examination prior to birth or fetal demise was accepted for analysis and related to obstetric outcome defined as gestational age at birth, birth weight and perinatal mortality. In cases of BFC III the venous velocimetry of 15 nonsurviving fetuses was compared to that of the 22 survivors. RESULTS: There was a significant correlation between venous blood velocity and placental vascular resistance. In the right hepatic vein there was a significant decrease in peak systolic and end-systolic velocities and an increase of maximum velocity during atrial contraction and pulsatility (P < 0.05). A decrease of all velocities and increase of pulsatility were noted in the ductus venosus (P < 0.05). A reversed flow in the ductus venosus was found in 9/37 fetuses and double umbilical venous pulsations in 16/37 fetuses. However, the hepatic vein seemed to be a better predictor of impending mortality than the ductus venosus. Changes in diastolic venous blood velocity and a double pulsation in the umbilical vein were closely related to perinatal mortality, although these parameters did not provide a useful threshold to optimize the timing of delivery. CONCLUSION: Diastolic venous velocimetry changes significantly in severely compromised fetuses. These changes might be of great clinical value in deciding on the timing of delivery to minimize damage to the fetus and newborn.     

Ultrasound-Guided Umbilical Venous Catheter Insertion With Alignment of the Umbilical Vein and Ductus Venosus

Previous studies have highlighted the importance of confirming the position of an umbilical venous catheter (UVC) tip by an ultrasound (US) examination. However, methods for preventing insertion into the portal circulation under US guidance have not yet been established. We report 15 cases in which a UVC was successfully passed through the ductus venosus by compressing the upper abdomen near the portal sinus of the liver to align the umbilical vein and ductus venosus under US guidance. The UVC was inserted into the correct position in 14 of the 15 neonates (93%) without complications.     

Reference ranges for fetal venous and atrioventricular blood flow parameters.

This cross-sectional study establishes reference ranges with gestation for Doppler parameters of fetal venous and atrioventricular blood flow. Color flow Doppler was used to examine 143 normal singleton pregnancies at 20-40 weeks' gestation. Flow velocity waveforms were recorded from the ductus venosus, right hepatic vein and inferior vena cava. The waveforms are triphasic, reflecting ventricular systole, early diastole and atrial contraction. Peak velocities for these parameters were measured with pulsed Doppler and a new index, the peak velocity index for veins (PVIV), was calculated. Similarly, time-averaged maximum velocities for the whole cardiac cycle were measured and the pulsatility index for veins (PIV) was calculated. Flow velocity waveforms were also recorded at the level of the atrioventricular valves and the ratios of peak velocities at early diastolic filling (E) and atrial contraction (A) were calculated. Regression analysis was used to define the association of each measured and calculated Doppler parameter with gestational age. Blood flow velocities in the fetal veins and velocities and E/A ratios across the atrioventricular valves increased significantly with gestation, whereas PVIV and PIV decreased. Blood flow velocities were highest in the ductus venosus and lowest in the right hepatic vein, and PVIV and PIV were highest in the hepatic vein and lowest in the ductus venosus. In the ductus venosus, there was always forward flow throughout the heart cycle, whereas in the inferior vena cava and hepatic vein during atrial contraction, flow was away from or towards the heart or there was no flow. Pulsatility of flow velocity waveforms in the venous system is the consequence of changes in pressure difference between the venous system and the heart during the heart cycle. The finding that PVIV and PIV decrease with gestation is consistent with decreasing cardiac afterload and maturation of diastolic ventricular function.     

Abnormalities of the fetal central veins and umbilico-portal system: prenatal ultrasonographic diagnosis and proposed classification.

OBJECTIVES: Anomalies of the fetal venous system are poorly documented and their pathogenesis is not well understood. The present study was undertaken to review the spectrum of fetal central veins and umbilico-portal system anomalies, and to propose a classification system. METHODS: A 7-year restrospective survey was conducted. RESULTS: Nineteen fetuses showed abnormal connection between central veins and the fetal heart. Three fetuses showed abnormal connections of the cardinal veins, two of which had interruption of the inferior vena cava, and one had isolated persistent left superior vena cava. Anomalies of pulmonary veins were seen in four fetuses: in two with asplenia syndrome, a vertical confluent pulmonary artery was observed. In a further two cases total anomalous pulmonary venous connections were found. Abnormalities of the umbilical vein (UV) were seen in 10 cases; seven had persistent right UV, and three had a spectrum of anomalies: One had abnormal connections of the UV to the left iliac vein associated with agenesis of the ductus venosus (DV) and hydrops fetalis. One case showed in utero occlusion of the DV by echogenic foci that resulted in a persistent left proximal UV and porto-systemic shunt. One case had obliteration of the DV secondary to in utero fetal hepatic fibrosis. Abnormalities of the vitelline veins or portal system were demonstrated in two cases. One had a left porto-systemic shunt which resolved spontaneously at 3 months of age, and one had secondary partial occlusion of the left portal system with liver echogenicities and direct communication of the UV with the right atrium. None of the 19 cases had an abnormal karyotype or evidence of in utero infection. CONCLUSIONS: Detection of various fetal vein anomalies in utero is feasible. The anomalies vary according to embryologic precursors or etiology. Two major mechanisms seem to be involved in the genesis of fetal vein anomalies: in most cases primary maldevelopment of the venous system occurs, while in the minority secondary anomalies from possible thromboembolic events or systemic disease may play a role.     

Two ductus venosus: a previously unreported anomaly

In early fetal life, the ductus venosus (DV) connects the umbilical vein, carrying nutrient-rich and oxygenated blood from the placenta to the inferior vena cava (IVC). We present the first ever case of a fetus with two ductus venosus. The fetus presented with a four-vessel cord and a dilated bowel at 31 weeks of gestation. Ultrasonography showed a persistent right umbilical vein. Echocardiographic investigation revealed normal cardiac anatomy with no major malformations. Two DVs with slightly different Doppler patterns were visualized anastomosing with the IVC. The baby was born uneventfully at 39 weeks of gestation with stable hemodynamics. Assuming every supernumerary umbilical vein should be connected to a DV for balanced circulation, a fetus with supernumerary umbilical veins lacking a corresponding number of DV connections is likely to be predisposed to complications such as hydrops fetalis and poor perinatal outcomes. The possibility of one or more umbilical veins lacking a DV connection warrants significant attention and regular monitoring from feto-maternal specialists, given the severity of the associated morbidity and mortality.     

Ductus venosus index: a method for evaluating right ventricular preload in the second-trimester fetus.

This study was designed to examine ventricular preload by measuring the ductus venosus index during the second trimester of pregnancy. A total of 137 women were entered into the study. Each fetus was examined with real-time, color and pulsed Doppler ultrasound. The color Doppler maximal velocity setting was adjusted so that the umbilical vein was homogeneous in color, did not demonstrate aliasing, and filled the venous lumen. The pulsed Doppler gate was placed within the ductus venosus in all subjects. Color Doppler identified a turbulent flow velocity within the ductus venosus which was not present in the umbilical vein, hepatic vein or inferior vena cava. The ductus venosus pulsed Doppler waveform demonstrated flow velocity from the umbilical vein to the heart during ventricular systole, the rapid filling phase of ventricular diastole, and atrial systole. However, flow velocity was decreased during atrial systole compared to ventricular systole and the rapid filling phase of diastole. The ductus venosus index was computed from the Doppler waveform of the ductus venosus at points consistent with ventricular and atrial systole ((ventricular systole - atrial systole)/ventricular systole). Regression analysis demonstrated a significant (p = 0.001) relationship between the ductus venosus index and gestational age (ductus venosus index = 75.5757 - 7.25484 x weeks gestation), standard error of the estimate = 7.21959; R = -0.451. One fetus with a hypoplastic left atrium and ventricle demonstrated a normal ductus venosus index. Two fetuses, one with pulmonary atresia and the second with severe cardiovascular dysfunction, demonstrated an abnormal ductus venosus index associated with absent flow velocity during atrial systole. This was associated with notching in the umbilical vein. The ductus venosus index is an angle-independent measurement from which right ventricular preload may be evaluated.     

Ductus venosus shunting in the fetal venous circulation: regulatory mechanisms, diagnostic methods and medical importance.

The fetal liver is located at the crossroads of the umbilical venous circulation. Anatomically, the ductus venosus (DV) and the intrahepatic branches of the portal vein are arranged in parallel. The actual DV shunting rate, i.e. the percentage of umbilical blood flow entering the DV measured by Doppler velocimetry, seems to be lower than that estimated using radioactively-labeled microspheres. In human fetuses the DV shunting rate is about 20-30%. Increases in the DV shunting rate are a general adaptational mechanism to fetal distress. Hypoxia results in a significant increase in the DV shunting rate, most probably in order to ensure an adequate supply of oxygen and glucose to vitally important organs such as the brain and heart. The mechanism of blood flow redistribution between the fetal liver and the DV is still a matter of debate. The isthmic portion of the DV contains less smooth muscle tissue than the intrahepatic branches of the portal vein, which in vitro react more forcefully in response to catecholamines than the DV.In growth-restricted human fetuses DV shunting is increased and the umbilical blood supply to the fetal liver is reduced. The long-term reduction of the hepatic blood supply may be involved in fetal growth restriction. The occlusion of the DV leads to a significant increase in cell proliferation in fetal skeletal muscle, heart, kidneys and liver, and possibly to an increase in insulin-like growth factor (IGF)-I and -II mRNA expression in the fetal liver. These findings hint at the possible role of the perfusion of the fetal liver in the control of the growth process.The quantification of DV shunting by Doppler velocimetry may improve the early recognition of fetal compromise in prenatal medicine. In this Review we summarize the published data on the anatomical structure and histology of the DV, the mechanisms of regulation of DV shunting, its role in fetal survival and growth and the possible use of the measurement of DV shunting in clinical practice.     

Foramen ovale: an ultrasonographic study of its relation to the inferior vena cava, ductus venosus and hepatic veins.

According to the literature, oxygenated blood from the ductus venosus and hepatic veins may either enter the right atrium before flowing through the foramen ovale to the left atrium, or flow directly from the ductus venosus and the hepatic veins to the foramen ovale, bypassing the right atrium. To address this problem, 103 normal fetuses were examined by two-dimensional imaging, M-mode and color Doppler at an average gestational age of 27 weeks (range, 15-40 weeks). The position of the ventricular septum and foramen ovale, and the angle and flow direction of the inferior vena cava, ductus venosus and hepatic veins were recorded. Two pathways for blood were described: a left ductus venosus-foramen ovale pathway that delivers blood directly to the foramen ovale circumventing the right atrium, and a right inferior vena cava-right atrium pathway that delivers blood into the right atrium through the right portion of the proximal inferior vena cava at an angle of 13 degrees to the long axis of the spine. The left and medial hepatic veins enter the left ductus venosus-foramen ovale pathway, and the right hepatic vein enters the right inferior vena cava-right atrium pathway. This supports the hypothesis that oxygenated blood from the ductus venosus and left hepatic veins flows directly through the foramen ovale to the left atrium avoiding extensive mixture in the inferior vena cava and an intermediate entrance to the right atrium.     

Doppler ultrasound measurements in the central circulation of anesthetized fetal sheep during obstruction of umbilical-placental blood flow.

OBJECTIVE: To establish by Doppler ultrasound the effects of acute blockage of umbilical cord vessels on the fetal central circulation. DESIGN: Experimental study in anesthetized pregnant sheep. METHODS: In 11 anesthetized pregnant sheep (0.66 of gestation), Doppler velocity profiles and/or venous flow rates were recorded in the ductus venosus, the fetal aorta, the umbilical arteries and the umbilical vein. This involved three phases. In Phase 1, the abdominal wall was closed (n = 9); Phase 2 was during fetoscopy after laparotomy (n = 8); Phase 3 was after coagulation of one of the umbilical arteries (n = 10). As an additional intervention in six fetuses, the umbilical cord was clamped for 5-15 s while blood flow velocity changes in the ductus venosus were observed. RESULTS: There were no significant differences between Doppler recordings in Phases 1 and 2. During Phase 3, the pulsatility index of the aorta and of the coagulated umbilical artery increased significantly. The umbilical vein blood flow rate was significantly reduced from a median of 408 (range, 243-575) mL/min/kg in Phase 1 to a median of 173 (range, 107-426) mL/min/kg in Phase 3, but the blood flow rate in the ductus venosus (median, 94; range, 56-148 mL/min/kg vs. median, 92; range, 33-237 mL/min/kg) remained unchanged. The ductus venosus/umbilical vein ratio increased from a median of 0.22 (range, 0.13-0.41) to 0.56 (range, 0.17-0.97) (P < 0.05). Compression of the cord (5-15 s) immediately reduced the time averaged maximum velocity in the ductus venosus from a mean of 51 (standard deviation, 11) cm/s to 20 (standard deviation, 6) cm/s (P < 0.001) without significant changes of the velocity profile. However, the pulsatility index increased from a mean of 0.55 (standard deviation, 0.19) to 1.89 (standard deviation, 0.73) (P < 0.001). CONCLUSION: Central venous flows in anesthetized fetal sheep are unaffected by laparotomy and hysterotomy. Obliteration of one umbilical artery increases the ductus venosus/umbilical vein volume flow (mL/min/kg) ratio. Compression of the umbilical cord shifts down blood flow velocity profiles in the ductus venosus, and the pulsatility index thus increases.     

Vascular anatomy of the fetus.

Real-time ultrasonography is utilized in this study to demonstrate most of the large vessels in fetuses from 22 weeks on. Images of the aorta, the coronary ostium, the vessels of the aortic arch, the carotids, the ductus arteriosus, the superior mesenteric artery, the celiac axis, the common iliac artery, the external iliac artery, and the femoral artery are demonstrated. Additionally, images of the subclavian vein, the superior mesenteric veins, the splenic vein, the portal vein, the hepatic veins, the ductus venosus, the renal veins, the iliac veins, and the inferior vena cava, as well as the pulmonary artery and veins and the azygos vein, are presented.     

Portal and umbilical venous blood supply to the liver in the human fetus near term.

OBJECTIVES: To determine the contribution of the umbilical (UV) and portal (PV) veins to blood supply to the human fetal liver in a low-risk population near term, and to assess the distribution between the left and right lobes. METHODS: In 91 low-risk pregnancies with normally grown fetuses at 36 weeks of gestation we measured the distribution of blood in the UV and PV to the right and left hepatic lobes using ultrasound imaging and Doppler techniques. RESULTS: The median (10(th), 90(th) centile) total UV return was 205 (127, 294) mL/min, of which 25% (13, 47%) was shunted through the ductus venosus, 55% (35, 66%) was distributed to the left hepatic lobe, and 20% (11, 30%) was distributed to the right hepatic lobe. While the left lobe was supplied exclusively by the UV, the right lobe received 50% (20, 70%; 37 (16, 65) mL/min) of its venous blood supply from the PV. The total venous blood supply to the liver parenchyma was 185 (114, 277) mL/min; 21% (8, 34%) came from the PV supply to the right lobe and the remainder came from the UV supply to both lobes. CONCLUSIONS: The venous supply to the left lobe is from nutrient-rich UV blood; for the right lobe, half is from UV blood and half from nutrient-poor PV blood. This watershed between the portal and umbilical venous flows to the fetal liver suggests a corresponding functional dichotomy; this may be modified by hemodynamic influences, with long-term consequences.     

Prenatal diagnosis of intrahepatic communications of the umbilical vein with atypical arteries (A-V fistulae) in two cases of trisomy 21 using color Doppler ultrasound.

We report on two cases of the prenatal diagnosis of arterio-venous communication between the intra-abdominal umbilical vein and atypical arteries. The diagnosis was made by color and spectral Doppler and 'color power angiography'. Both cases presented with hydrops fetalis, one at 14 and the other at 31 weeks of gestation. In the first case, color Doppler demonstrated an atypical arterial vessel connecting the umbilical vein with the aorta; the ductus venosus was patent. Echocardiography showed a so-called atrioventricular canal. In the second case, a complex intrahepatic vascular malformation was found. Color Doppler demonstrated communications between the umbilical vein and the hepatic artery and an atypical artery; the ductus venosus was patent. In the latter case polyhydramnios, duodenal atresia and macroglossia were additionally detected. In both cases, fetal karyotyping revealed trisomy 21. The first case resulted in a missed abortion, the second in a stillbirth. All findings were confirmed on autopsy. Of interest is that both fetuses were affected with trisomy 21. The increasing use of color Doppler in prenatal diagnosis, especially in hemodynamically compromised fetuses, will help to determine the actual incidence of complex vascular malformations of the umbilical vein and to elucidate the impact of such malformations on fetal outcome.     

Aberrant Drainage of the Umbilical Vein Into the Coronary Sinus Without Ductus Venosus Agenesis

We describe a case series of 4 fetuses with ectopic connections of the ductus venosus to the coronary sinus detected prospectively between August 2011 and February 2012 in 2 congenital cardiologic centers. An enlarged coronary sinus alerted the sonographer. Fetal echocardiography showed ectopic connection of the ductus venosus in an enlarged coronary sinus in all 4 cases. To our knowledge, this anatomic form of ectopic umbilical vein drainage has not previously been reported. The infants were doing well. This venous variant should be considered in cases of isolated coronary sinus dilatation after elimination of a left superior vena cava and a totally anomalous pulmonary vein connection.