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;     

胎儿静脉导管闭锁产前超声表现

目的 观察静脉导管(DV)闭锁胎儿产前超声表现。方法 回顾性分析20胎DV闭锁胎儿产前超声表现，记录妊娠结局。结果 20胎DV位置及走行均正常，其中16胎(16/20,80.00%)DV管壁呈等回声，3胎(3/20,15.00%)管壁呈条索状强回声，1胎(1/20,5.00%)DV管壁回声增强，其内为低回声，呈“双轨”征；CDFI均未见DV内存在血流信号。3胎(3/20,15.00%)合并其他结构畸形，包括2胎永存右脐静脉(其中1胎染色体异常)和1胎肺动脉交叉。19胎(19/20,95.00%)出生后证实为DV闭锁，出生后新生儿Apgar评分、体质量及随访均未见异常；1胎(1/20,5.00%)死于宫内，经尸检确定DV闭锁。结论 胎儿DV闭锁产前超声可见管壁呈等回声、条索状强回声或“双轨”征，腔内无血流信号。     

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。     

Human and software error in ductus venosus Doppler waveform analysis

Ductus venosus waveform analysis has become increasingly affected by technical errors; however, these errors could be avoided if more attention was paid during sampling and analysis. The most common misevaluations include incorrect tracing, under- or overestimation of the peak systolic velocity, overestimation of the end-diastolic velocity, and, as a consequence, incorrect calculation of the pulsatility index facilitated either by human or software error. This article proposes practical suggestions to avoid technical errors in ductus venosus waveform analysis.     

新生儿静脉导管血流动力学与肝功能的相关性研究

【】目的 探讨不同胎龄新生儿静脉导管血流动力学指标与肝功能的关系。方法 选择确诊为静脉导管未闭的新生儿175例作为病例组,并依据胎龄分为A组(55例,胎龄31～34周)、B组(58例,胎龄35～37周)、C组(62例,胎龄38～40周),于出生后2～3天应用彩色多普勒超声检测各组新生儿静脉导管内径、平均血流速度并计算血流量;选择确诊为静脉导管闭合的新生儿60例作为对照组(胎龄38～40周);观察各组肝功能指标[谷丙转氨酶(ALT)、谷草转氨酶(AST)、总胆汁酸(TBA)、血氨(NH3)、血清总蛋白(TP)]及凝血酶原时间(PT)的变化。结果 A组的静脉导管血流量明显大于B组及C组,B组的静脉导管血流量大于C组,三组间差异有统计学意义(P<0.01);A组的ALT、AST高于B组、C组及对照组(P<0.01),但B组、C组与对照组间比较差异无统计学意义(P>0.05);A组的TBA、NH3及PT均高于B组及C组,A组的TP低于B组及C组,三组间差异有统计学意义(P<0.01),但C组的TBA、NH3、TP、PT与对照组比较差异均无统计学意义(P>0.05)。结论 新生儿胎龄越小,未闭合的静脉导管血流量越大,其所引起的较大量门体静脉分流会对肝功能产生影响,而且静脉导管分流量越大,对新生儿肝功能影响越明显。     

产前超声诊断静脉导管缺失及走行异常

目的 探讨胎儿静脉导管(DV)缺失及走行异常的产前超声图像特征及其临床意义。方法 回顾性分析本院诊断为胎儿DV缺失及走行异常9胎的临床及声像图特征。结果 7胎DV缺失,脐静脉直接回流至右心房伴静脉导管缺失;2胎DV走行异常,1胎汇入冠状静脉窦,1胎汇入肝静脉。胎儿结局:5胎因合并畸形引产,1胎生后由于膈疝缺口太大及肺发育不良死亡,1胎28周早产死亡,2胎生后暂未见明显异常;8胎合并其他畸形,1胎孤立性DV走行异常;7胎合并心内畸形,3胎合并心外畸形。结论 产前超声检查可诊断DV缺失及走行异常;应结合畸形严重程度及染色体检查结果评价预后。     

Prenatal sonography of extracorporeal ductus venosus in association with large fetal gastroschisis

Liver herniation commonly associated with omphalocele occurs in only approximately 2.3% to 16% of fetuses with gastroschisis. Liver herniation in such cases is associated with considerably decreased survival rates (43% vs 97% with or without liver herniation, respectively). Rarely, abnormally positioned fetal hepatic vasculature has been reported mainly in association with congenital diaphragmatic hernia. In these rare cases, intrathoracic depiction of hepatic venous vasculature has assisted in confirming intrathoracic displacement of the fetal liver. We present a case of a large gastroschisis with complete herniation of the fetal liver in which prenatal sonography depicted an extracorporeal ductus venosus.     

胎儿静脉导管异位引流至冠状静脉窦产前超声表现:3例报告及文献复习

目的 结合文献及3例临床所见,观察胎儿静脉导管（DV）异位引流至冠状静脉窦（CS）产前超声表现。方法 回顾性分析3胎DV异位引流至CS胎儿,结合文献复习观察其产前超声特征。结果 产前超声于3胎四腔心后切面均见CS扩张,内径分别为3.6、8.2及4.8 mm;DV异位引流至扩张的CS。于1胎（序号1）见4支肺静脉汇入左心房（LA）,DV经房间隔后方于左心房顶绕行至扩张的CS;1胎（序号2）三血管气管切面肺动脉主干左侧见永存左上腔静脉（PLSVC）,旋转探头追踪观察发现DV与CS相连;1胎（序号3）4支肺静脉均回流入LA,三血管气管切面见主动脉弓位于气管右侧、左锁骨下动脉起自降主动脉起始部。结论 产前超声观察胎儿DV异位引流至CS四腔心后切面可见CS扩张,并可合并其他血管畸形。     

Second-trimester ductus venosus measurement and adverse perinatal outcome in fetuses with congenital heart disease.

OBJECTIVE: The purpose of this study was to determine whether Doppler velocimetry of the ductus venosus (DV) predicts adverse perinatal outcome in congenital heart disease (CHD). METHODS: We conducted a retrospective cohort study of all pregnant women undergoing fetal echocardiography for CHD in a single perinatal center during a 2-year period. We compared outcomes for fetuses having a diagnosis of CHD in the second trimester and abnormal DV Doppler velocimetric findings with those having CHD and normal DV Doppler findings. Karyotype, gestational age at delivery, fetal loss rate, and rate of termination were assessed. The referral value for an abnormal DV pulsatility index was above the 95th percentile for gestational age. Statistical analysis included the t test, Fisher exact test, and chi(2) test. RESULTS: The incidence of CHD in our population was 7%. There were 98 patients with CHD; of those, 31 had DV measurement. A total of 9 patients had an abnormal DV. Three of this group (33%) had intrauterine fetal death or perinatal death. In patients with CHD and normal DV measurements, 83% had living children versus 33% in the group with an abnormal DV (P < .05). There was no statistically significant difference in the rate of aneuploidy between the normal DV (15%) and abnormal DV (20%) groups (P = .65). The mean gestational age at delivery was similar between the normal (37.63 weeks) and abnormal (38.33 weeks) DV groups (P = .71). There was no difference in the rate of pregnancy termination. CONCLUSIONS: Abnormal second-trimester DV measurements are predictive of adverse perinatal outcome in patients with CHD, independent of karyotype or gestational age at delivery. This information may have a role in the counseling of parents with CHD.     

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.     

静脉导管超声检测在产前检查中的应用价值

本文着重介绍超声检测静脉导管在产前检查中的应用价值,阐述静脉导管的组织解剖结构、静脉导管分流的机制以及静脉导管在诊断胎儿生长受限、先天性心脏病等疾病中的临床意义.     

Doppler ultrasonographic evaluation of ductus venosus blood flow in 55 canine fetuses

Purpose

The ductus venosus (DV) blood flow has been studied in fetal lambs and in humans. This study aims to describe the velocities, the Doppler indices and the morphological patterns of the venous blood flow in the DV of canine fetuses during physiological pregnancy.

Methods

The DV of 55 canine fetuses has been evaluated and the waveforms described using B-mode, color and pulsed-wave Doppler ultrasound technology.

Results

We found 48 diphasic waves and 7 threephasic waves. No monophasic waveform was found. Six of seven threephasic waveforms belonged to litters in which perinatal mortality occurred. The peak velocity during ventricular systole S (cm/s), the peak velocity during the ventricular diastole D (cm/s), the velocity during atrial contraction aV (cm/s), the S/D index, the pulsatility index (PI) and the resistance index were measured.

Conclusions

All Doppler indices and velocities were significantly correlated with each other (p < 0.05). The number of newborn puppies and the age of bitches were not related to DV vascular indices or flow rate (p > 0.05). Gestational age was proportional to the PI (p < 0.02). Doppler ultrasonography allows the assessment of DV blood flow in canine fetuses during pregnancy.     

Middle cerebral artery peak systolic and ductus venosus velocity waveforms in the hydropic fetus.

OBJECTIVE: The purpose of this study was to assess whether Doppler assessment of the middle cerebral artery (MCA) peak systolic velocity (PSV) and ductus venosus (DV) velocity waveforms during sonography of hydropic fetuses may specify the cause of fetal hydrops. METHODS: A level II sonographic examination was performed in 16 hydropic fetuses, and the MCA PSV and DV velocity waveforms were assessed. The MCA PSV values divided hydropic fetuses into anemic (group 1) and nonanemic (group 2) fetuses. In group 2 fetuses, the DV was defined as normal or abnormal. Sonographic examination and Doppler assessment of these vessels specified the cause of hydrops and indicated the use of specific investigations for diagnosing the etiology of fetal hydrops. RESULTS: Seven of 16 fetuses had MCA PSV values greater than 1.50 multiples of the median (group 1). Nine of 16 fetuses had normal MCA PSV values (group 2); among them, 7 of 9 had either absent or reversed flow in the DV, and 2 had a normal DV. In group 1, the cause of fetal anemia was investigated by maternal serum tests, and 5 cordocentesis procedures were performed. In group 2, 7 of 9 fetuses had reversed flow in the DV, which suggested a cardiac abnormality confirmed by echocardiography. Five cordocentesis procedures were performed for fetal karyotype, and in 2 fetuses, the cause of hydrops was idiopathic. CONCLUSIONS: Our data suggest that assessment of the MCA PSV and DV velocity waveforms in the hydropic fetus may further our knowledge of the etiology of hydrops and may indicate which investigations among the many available should be used for diagnosing the cause of fetal hydrops.     

Correlation between ductus venosus spectrum and right ventricular diastolic function in isolated single-umbilical-artery foetus and normal foetus in third trimester

BACKGROUNDSingle umbilical artery (SUA) is the most common umbilical cord malformation in prenatal diagnosis. The presence of an SUA can cause blood circulation disorder in the foetus and functional changes of the foetal heart, affecting foetal circulation. The right ventricular diastolic functions in foetuses with isolated SUA and in normal foetuses in the third trimester were evaluated using the spectral Doppler of blood flow in the foetal ductus venosus (DV).AIMTo evaluate the right ventricular diastolic functions in foetuses with isolated SUA and in normal foetuses in the third trimester.METHODSColour Doppler was used to measure the spectrum of foetal DV and tricuspid orifice in 34 foetuses with isolated SUA aged 28-39 wk and in age-matched healthy controls. The DV flow velocities and velocity ratios were measured. The early passive/late active (E/A) ratio at the tricuspid orifice and tissue Doppler Tei index of the foetal right ventricular in the two groups were also measured.RESULTSDuring the third trimester, the isolated SUA group showed a lower ‘a’-wave peak velocity in the DV than the control group (P < 0.05). The correlations between the velocity ratios and E/A ratio at the tricuspid orifice in the two groups were analysed, and the correlation between the ventricular late diastolic velocity/ventricular diastolic peak flow velocity and E/A ratios was the best (R² of the isolated SUA group: 0.520; R² of the control group: 0.358). The correlations between the velocity ratios and tissue Doppler Tei index of foetal right ventricular in the two groups were analysed, and the correlation between the pulsatility index for veins (PIV) and tissue Doppler Tei index ratios was the best (R² of the isolated SUA group: 0.865; R² of the control group: 0.627).CONCLUSIONIn the isolated SUA group, the atrial systolic peak velocity ‘a’ decreased, and this finding might be related to the changes in foetal cardiac functions. The ratio of ventricular late diastolic velocity to ventricular diastolic peak flow velocity was closely related to the E/A ratio at the tricuspid valve and can be used to identify changes in the right ventricular diastolic functions of isolated SUA and healthy foetuses. PIV was closely related to the tissue Doppler Tei index of the foetal right ventricular and can be used to identify the right ventricular overall functions of isolated SUA and healthy foetuses.     

中晚孕期正常胎儿静脉导管血流动力学研究

目的 建立中晚孕期正常胎儿静脉导管多普勒血流参数参考范围。方法 应用彩色多普勒超声检查138例16-41孕周正常单胎胎儿。在静脉导管入口处记录静脉导管血流波形，测定血流参数包括：心室收缩期峰值流速（S）、心室舒张期峰值流速（D）、心房收缩期最大流速（A）、平均流速（Vmean）、S／A值、S／D值、（S-A）／S值、（S-A）／D值和（S-A）／Vmean值。结果 正常胎儿静脉导管的血流参数S、D、A、Vmean随着孕龄的增加而增加（均P〈0．01或P〈0．05）；而S／A、（S-A）／S、（S-A）／Vmean和（S-A）／D值随着孕龄增加而减低（均P〈0．01或P〈0．05），S／D与孕龄相关性差（r=0．1，P〉0．05）。结论 正常胎儿静脉导管血流波形呈特殊的三相波形，血流速度随着孕龄增加而增加，而阻力指数随着孕龄增加而减低，S／D值在妊娠中持续不变。     

Ductus venosus blood flow resistance and congenital heart defects in the second trimester

PURPOSE: To study the association between fetal cardiac defects and the presence of abnormal blood flow resistance of the ductus venosus during the second trimester of pregnancy. METHODS: This retrospective case-control study included 72 pregnancies with fetal cardiac anomalies and 267 normal pregnancies. In fetuses with cardiac anomalies, Doppler velocimetry resistances of the ductus venosus were serially determined prior to birth. The Doppler velocimetry resistances obtained from fetuses with cardiac anomalies were in turn compared with median values derived from 267 normal pregnancies to compensate for biometric bias due to gestational age. RESULTS: Among the 72 pregnancies with fetal cardiac anomalies, 26 fetuses showed isolated congenital heart disease (CHD) without nonimmune fetal hydrops (NIFH) (group A), 10 fetuses showed isolated CHD with severe heart defects, including atrioventricular valve insufficiency and consecutive cardiogenic NIHF (group B), and 36 fetuses showed chromosomal abnormalities, nonchromosomal extracardiac malformations, noncardiogenic NIHF, and fetal growth restriction summarized as nonisolated CHD (group C). Based on the pulsatility index for the vein of the ductus venosus, the area under the receiver operating characteristic (ROC) curves was 0.71, 0.73, and 0.86 for groups A, B, and C, respectively. CONCLUSION: In the 36 fetuses from group C, increased pulsatility index for vein of the ductus venosus (DVPIV) yielded a significant area under the ROC curve (0.86) with a sensitivity of 0.78 and a specificity of 0.78. Increased DVPIVs during the second trimester of pregnancy are highly correlated with fetal cardiac anomalies associated with chromosomal and extracardiac anomalies.     

正常孕10～19周胎儿静脉导管彩色血流频谱的初步探讨

目的探讨经腹彩色多普勒超声获取10～19周胎儿静脉导管血流频谱的正确采样及检测方法并建立其阻力指数（RI）、搏动指数（PI）、心室收缩期峰值流速与心房收缩期最低流速比值（S／A）的正常参考值范围；探讨生理性零或反向房收缩（RA）波在正常10～14周胎儿静脉导管中的发生率。方法随机选取111例孕10～19周的正常单胎孕妇，在不校正测量角度的情况下进行胎儿静脉导管血流频谱检测，对血流参数RI、PI和S／A值与孕周进行相关和回归分析。结果静脉导管的血流参数值随着胎龄的增加而减低，其95％的参考值范围分别为R10．34～0．90、P10．21～1．32和S／A1．23～6．65；10～14周胎龄段的50例胎儿静脉导管血流频谱中未见零或RA波。结论应用经腹彩色多普勒超声对10～19周胎儿静脉导管进行血流动力学评价方法简单，结果准确，容易掌握；静脉导管血流参数RI、PI和S／A值随着胎龄的增加而减低，其参考值范围可进一步应用于对胎儿染色体畸形和严重心脏畸形的研究；10～14周正常胎儿静脉导管内是否存在生理性零或RA波有待于进一步研究。     

Stenosis of Alternative Umbilical Venous Pathways in Absence of the Ductus Venosus

Objective. We evaluated fetuses with absence of the ductus venosus (ADV) and restricted alternative umbilical venous pathways. Methods. We identified 3 cases that fit our objective. The angles of insonation for spectral Doppler ultrasound interrogation were less than 20° in all cases. We used commercially available ultrasound systems with a curved array transducer. Results. In all 3 cases, we noted mild cardiac volume overload without fetal hydrops. Conclusions. We speculate that the fetus with ADV and a restrictive alternative umbilical venous pathway may have a more benign clinical course than fetuses previously reported with unrestricted alternative pathways.