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The Human Fetal Venous System
Normal Embryologic, Anatomic, and Physiologic Characteristics and Developmental Abnormalities
Sozos J. Fasouliotis, MD, Reuven Achiron, MD, Zvi Kivilevitch, MD, Simcha Yagel, MD
Objective. The introduction of high-resolution ultrasonography combined with color-coded Doppler imaging offered a breakthrough in the evaluation of the human fetal venous system, considerably enhancing our understanding of fetal venous circulation in normal physiologic conditions, as well as providing us the ability to study circulatory changes in abnormal circumstances. The purpose of this study was to describe the normal anatomic development and complex of anomalies of the human fetal venous system and to review recently published series of these anomalies. Methods. Normal embryologic and anatomic development is described. An English language literature search of recent MEDLINE listings was performed to glean data from recently published series reporting prenatal diagnosis of the various anomalies and their associated malformations. Results. Anomalies of the human fetal venous system occur sporadically, often associated with cardiac or other malformations. The pathophysiologic mechanisms leading to abnormal in utero development of the human venous system remain largely undetermined. On the basis of the type of vein involved, embryologic precursor, and etiologic correlation (primary or secondary), classification into 4 major groups is described. Conclusions. Prenatal evaluation of fetuses found to have anomalies of the venous system should include a careful search for cardiac anomalies, including pulmonary venous drainage, and a detailed anatomic survey of the umbilical, portal, hepatic, and ductal systems to determine aberrant communication and, if possible, to discover clues to systemic diseases or thromboembolic phenomena. Key words: anomalies; fetal venous system; prenatal diagnosis.
Abbreviations PAPVC, partial anomalous pulmonary venous connection; TAPVC, total anomalous pulmonary venous connection Received March 28, 2002, from the Department of Obstetrics and Gynecology, Hadassah University Hospital, Ein Kerem, Jerusalem, Israel (S.J.F.); Department of Obstetrics and Gynecology, Sheba Medical Center, Tel Hashomer, Jerusalem, Israel (R.A.); Macabbi Health Services, Ultrasound Center, Beer Sheba, Israel (Z.K.); and Department of Obstetrics and Gynecology, Hadassah University Hospital, Mount Scopus, Jerusalem, Israel (S.Y.). Revision requested April 9, 2002. Revised manuscript accepted for publication July 1, 2002. Address correspondence and reprint requests to Simcha Yagel, MD, Department of Obstetrics and Gynecology, Hadassah University Hospital, Mount Scopus, PO Box 24035, Mount Scopus, Jerusalem, Israel.
ecent developments in ultrasonographic imaging, including the development of highresolution ultrasonography combined with color-coded Doppler imaging, facilitate prenatal diagnosis of fetal malformations of varying severity. This in turn enables caregivers to provide a prognosis to the parents depending on the malformation detected. Furthermore, prenatal diagnosis of fetal malformations, especially of the heart, has been found to improve morbidity and mortality rates during postnatal treatment.1–3 Although the fetal venous system was described almost 20 years ago,4,5 it was the introduction of highresolution ultrasonography combined with colorcoded Doppler imaging that offered a breakthrough in the evaluation of this system,6–8 considerably enhanc-
© 2002 by the American Institute of Ultrasound in Medicine • J Ultrasound Med 21:1145–1158, 2002 • 0278-4297/02/$3.50
22–24 prenatal treatment of these patients may become problematic. whereas the left anterior cardinal vein disappears. the entire right umbilical vein and proximal segment of the left umbilical vein atrophy and soon disappear.9–16 Improvement in imaging technology led to a search for the in utero diagnosis of other rare anomalies of the fetal venous inlet system. and prognosis of these abnormalities is derived from neonates19–21.The Human Fetal Venous System ing our understanding of fetal venous circulation in normal physiologic conditions as well as providing us the ability to study circulatory changes in abnormal circumstances. whereas the hepatic vein and ductus venosus drain into the infracardiac portion of the inferior vena cava (Fig. 1B). and understanding the embryologic basis and pathophysiologic mechanisms of fetal venous inlet system abnormalities becomes increasingly important for accurate prenatal counseling. in the 9-mm embryo. with the anterior and posterior cardinal veins draining the embryonic cranial and caudal parts of the body. the pathophysiologic mechanisms leading to in utero abnormal development remain largely undetermined. First the developing hepatic sinusoids link to both vitelline veins and then tap the umbilical vein at day 32. 1C). Laterally expanding primitive left and right lobes of the liver contact the paired umbilical veins coursing close by. thus forming an anastomotic system among liver sinusoids. respectively. Thus all placental blood enters the right atrium through the left distal umbilical vein. in the 6-mm embryo. The umbilical veins also undergo several changes. As development continues. all of which open to the right and left horns of the sinus venosus of the heart. The cardinal veins form the main venous drainage system of the embryo body. Nevertheless. Any 1146 further development of the fetal venous system represents changes occurring in these symmetric venous systems (Fig. Each vitelline vein is then interrupted by the sinusoidal labyrinth to a distal segment extending from the yolk sac to the liver. 1A). After the atrophy of the posterior cardinal veins. importance. causing the loss of connection between both umbilical veins with the fetal heart. however. after division J Ultrasound Med 21:1145–1158. whereas the left proximal and right distal vitelline veins atrophy and disappear (Fig. Eventually. fetal outcome cannot be extrapolated from data obtained from neonates with similar abnormalities. inferior vena cava. the left and right portal veins are already part of the left umbilical vein.16. which is the third venous system entering the sinus venosus of the primitive heart.6 The most commonly reported abnormalities involve the intrahepatic umbilical vein. In the 5-mm embryo. which includes the umbilical veins from the chorion. In this review we provide a detailed description of the embryologic. and pulmonary veins. emptying into the left horn of the sinus venosus. and proximal right vitelline vein. ductus venosus. in which the right proximal stem represents the hepatic vein. the subcardinal and supracardinal veins are formed. through left-to-right anastomosis.18 The only information currently available regarding the etiology. Normal Development of the Human Fetal Venous System Embryologic Characteristics A system of 3 paired veins is found in the 4-week embryo. the course of the umbilical vein. which is finally converted into the portal vein and to a proximal segment extending from the liver to the heart. anatomic. which then empty into a common cardinal vein. as well as associated anomalies. The left brachiocephalic vein is formed during the eighth week from the right anterior and right common cardinal veins. portal and hepatic veins. we describe a classification system of associated developmental abnormalities of this system based on the type of vein involved. As the number of case reports regarding various fetal vein abnormalities increases.13–20 Two recent relatively large series describing fetal venous system malformations suggested that in a targeted fetal organ scan. and physiologic characteristics of the normal human fetal venous system.14. and the cardinal veins from the body of the embryo itself. The fetal liver and its development in the septum transversus play an important role in modifying the primitive vitelline and umbilical systems into their final shape. and umbilical veins. ductus venosus. vitelline veins. the left umbilical vein becomes the dominant conduit of blood from the placenta. but rare cases of agenesis of the ductus venosus have also been reported. The latter. which bypass the liver sinusoids. In addition. can be clearly visualized and accurately diagnosed with the use of two-dimensional gray scale and color Doppler imaging. 2002 . the vitelline veins from the yolk sac.
which joins the ductus venosus to the heart (the hepatocardiac portion. and bulbus cordis (BC). which is noticeably larger than the right. As the atrial cavity develops. From this vessel arise the portal vein. PA. pulmonary artery. From this same “twist. Posterior view of the mature venous system. the superior mesenteric vein. Modified with permission from the McGill Molson Medical Informatics Project (http://sprojects. until finally the 2 right and left branches of the pulmonary stem enter the atrial cavity. A. 1C). 2002 Figure 1. arising from the vitelline vein within the liver (hepatic sinusoids). C. larger than the right portal vein. the stem of the pulmonary vein is gradually incorporated into the left atrial wall.html). form the azygos and hemiazygos veins above this level. The umbilical vein and the left portal vein maintain the same diameter. This situation is reversed in the adult after atrophy of the umbilical vein and the ductus venosus. The ductus venosus is unique to fetal life. the prerenal segment from the right subcardinal vein. which joins distally with the left hepatJ Ultrasound Med 21:1145–1158. Its intrafetal portion ascends by way of the falciform ligament to join the umbilical segment of the left portal vein. and the hepatic segment from the proximal vitelline vein and the liver sinusoids. IVC indicates inferior vena cava. B. In the human embryonic venous system. Fig.ca/embryology/cvs/default.mcgill.” arises the ductus venosus. 1147 . umbilical veins (UV). Posterior view of the changes formed with the goal of introducing right-sided asymmetry in the adult.Fasouliotis et al in the renal area. and SVC. the umbilical vein transfers blood from the placenta to the fetus. and the intrathoracic inferior vena cava. truncus arteriosus (TA).mmi.” known as the “pars transversa. There is therefore a marked preference in both the quality and quantity of blood flowing through the left lobe of the liver. × indicates atrophied vessels. The upper segments of the inferior vena cava are derived from the subsupracardinal anastomosis at the renal area. sinus venosus (SV). superior vena cava. whereas below this level the left supracardinal vein degenerates. and the right supracardinal vein becomes the caudal part of the inferior vena cava. which receives blood primarily recycled through the main portal vein. Anatomic Characteristics By the end of the first trimester. The common pulmonary vein can be identified in a 4-mm embryo as an invagination of the dorsal wall of the left atrium. the left umbilical vein eventually remains the only vein from the placenta to the fetal heart. and the development of its connection and the ductus venosus (“the critical anastomosis”) is of primary importance in the development of the venous drainage system and supply of oxygenated blood to the fetus. The left portal vein joins the anterior and posterior branches of the right portal vein through an almost 90° right turn. Posterior view of the paired vitelline veins (VV).
Figure 2. gallbladder. and inferior vena cava facilitate the transfer of blood with the highest possible oxygen concentration to the fetal heart. hepatic veins. the pathophysiologic mechanisms controlling the function of the human fetal venous circulatory system are only partially elucidated. and the color indicates the degree of oxygen content (red. LPV. and blue. inferior vena cava. we have classified them into 4 major groups. low). Examination of the effect of fetal breathing movements on the circulation in the venous system revealed that changes in the pressure gradient between the intraabdominal and intrathoracic cavities during breathing movements caused subsequent changes in blood flow in the venous system. these changes may cause an additional negative effect on venous return and flow in the umbilical vein. resulting in a drop in the end-diastolic volume of the arteries on the one hand and corresponding decline (through the Frank-Starling mechanism) in stroke volume of the heart on the other side of the system. Table 2 summarizes this classification system. A decrease in venous return causes a drop in placental bed drainage and filling of the heart ventricles. on the basis of the type of vein involved. Specifically. Table 1 summarizes the correlation of embryonic structures in the venous and arterial systems with the equivalent adult anatomy. Reprinted by permission from Ultrasound in Obstetrics and Gynecology. 2002 . Schematic representation of the fetal umbilical. portal.28. foramen ovale. 2). Physiologic Characteristics Principal vessels such as the ductus venosus. Developmental Abnormalities of the Human Fetal Venous System We speculate that the normal development of the fetal venous system may be disturbed by either of 2 different events: (1) primary failure to transform or to form the critical anastomosis and (2) secondary occlusion of an already transformed system. hepatic veins. EPV. portal sinus. which have a direct effect on venous return to the heart.16 1148 J Ultrasound Med 21:1145–1158. and UV. right portal vein. FO. whereas during expiration the opposite events occur.25–27 as does the question of oxygen concentration–dependent anatomic nervous control of the activity of this sphincter. as well as systolic and diastolic blood flow in the arteries. PS. IVC. RA.29 Other studies showed that changes in intrathoracic pressure caused by fetal breathing movements affect both venous return to the heart and the arterial system. However. medium. GB.14 The existence of a “sphincter” that regulates blood flow through the ductus venosus to the heart remains a subject of debate. during inspiration the pressure gradient rose from approximately 0 to 3 to approximately 22 mm Hg. This intimate link between the function of the venous vascular system and the other elements of the fetal circulation is shown physiologically during fetal breathing movements.14 Low placental resistance and improved cardiac contraction aid in the creation of a pressure gradient between the atria and ventricles that reduces the preload in the venous circulatory system and allows blood to flow toward the heart. extrahepatic portal vein. purple. left portal vein. umbilical vein.The Human Fetal Venous System ic vein and the inferior vena cava just proximal to the entrance into the right atrium (Fig. RPV. embryologic precursor. Thus. HV. causing an increase in the pressure gradient between the umbilical vein and the thoracic part of the ductus venosus and leading to a rise in flow velocity in the umbilical vein. and hepatic venous circulations. The arrows indicate the direction of blood flow. right atrium. As a result. and etiologic correlation (primary or secondary). DV indicates ductus venosus.30 Changes in venous return to the fetal heart can also influence emptying of the placental bed. high.
portion within septum transversum contributes to hepatic portal system Degenerates proximally and distally. These mainly result from primary failure to form the critical anastomosis.mcgill. This raised the possibility that the finding was largely attributable to some other cause. the placenta venous return is rerouted to the systemic veins. venous blood flow from the placenta is blocked. and degeneration of both umbilical veins occurs in early embryonic life. however. cases without notable cardiac involvement in which fetuses may survive have been reported. anastomoses with right anterior cardinal vein distally to form left brachiocephalic vein Oblique vein of left atrium Disappears Coronary sinus Superior vena cava Superior vena cava Azygos vein Ligamentum venosum Ligamentum teres distally.ca/embryology/cvs/default. anastomosis between the umbilical and vitelline veins fails.mmi.33 It is in these cases that accurate prenatal diagnosis is essential to provide appropriate counseling to the parents. Hence.Fasouliotis et al Table 1. the bloodstream that forms the caudal part of the body reaches the heart by way of the azygos vein and superior vena cava. it may be concluded that in a case of primary failure to form these critical anastomoses. distal portion contributes to left superior intercostal vein.” According to this theory. Apart from the complex heterotaxy syndromes. through unidentified causes of embryologic maldevelopment. portion within septum transversum contributes to hepatic portal system Fuses with right vitelline vein to form portal vein Modified with permission from the McGill Molson Medical Informatics Project (http://sprojects.13.html). The persistent left superior vena cava is another example of primary failure to create anastomosis and to form the left brachiocephalic vein. Another study showed that in a 7-mm pig embryo there was a connection between the primitive pelvic venous plexus that drains the lower limb bud and the embryonic umbilical vein. Primary failure to form the critical anastomosis may involve the iliac vein.32 Most of these syndromes are associated with complex anomalies. which leads to the opening of vascular channels through anastomosis to the supracardinal components of the inferior vena cava. Instead.22. Abnormal Connection of the Cardinal Veins Heterotaxy syndromes represent a pure example of abnormal primary development of the venous system. Fetal Venous Structures and Their Adult Equivalent Vessels Embryologic Structure Adult Equivalent Left anterior cardinal vein Left common cardinal vein Left posterior cardinal vein Left horn of sinus venosus Right anterior cardinal vein Right common cardinal vein Right posterior cardinal vein Ductus venosus Left umbilical vein Right umbilical vein Left vitelline vein Disappears proximally. White et al35 offered probably the most convincing explanation of an embryologic basis for this rare anomaly. with an aberrant vessel J Ultrasound Med 21:1145–1158. with a mouse model existing that explains the transmission of situs abnormalities in polysplenia-asplenia syndromes.31. it shunts its blood directly into the right supracardinal vein. 2002 shunted between the placenta and the systemic veins. perhaps a congenital generalized lymphangiectasia as manifested in Noonan syndrome. especially cardiac malformations that influence the prognosis appreciably. isolated anomalies of the cardinal veins have also been reported.34 Several of these cases with an abnormal umbilical vein connection were reported to be associated with Noonan syndrome. As a result. Hydrops fetalis was a common finding in these fetuses. although the severity of the edema was out of proportion to the vascular anomaly.36 Summarizing the results of these studies. degenerates proximally. Absence of the inferior vena cava with azygos continuation results from primary failure of the right subcardinal vein to connect with the hepatic segment of the inferior vena cava. Abnormalities of the Umbilical Veins Abnormalities of the portal and umbilical veins form the major group of fetal venous anomalies. introducing the term “critical anastomosis.21. the superior 1149 .
Umbilical veins a. Two main paths have been described.38 According to the findings. The first includes fetuses in which the umbilical flow entirely bypasses the liver. 4).44 Several pathophysiologic explanations for these aberrations have been proposed. causing reopening of this part of the umbilical vein and resulting in a persistent proximal left umbilical vein. Proposed Classification System for Abnormal Connections of the Fetal Venous System With the Heart A. partial failure to form the critical anastomosis may be more common.The Human Fetal Venous System Table 2. Fig. many reported cases had an abnormal connection of the umbilical vein to the iliac vein.41. an adequate connection between the umbilical vein and the vitelline venous system was established. 2 subgroups may be distinguished. oxygenated blood coming from the placenta is diverted into the already closed proximal left umbilical vein. Partial agenesis of right or left portal branch (portosystemic shunt) D. much less common path involves the direct entry of the umbilical vein into the right atrium. and Noonan syndrome.40 This anomaly is characterized by failure of the right umbilical vein to form anastomosis with the right vitelline vein and degeneration of the left umbilical vein. Complex malformations.16.45 Echogenic foci found within the fetal liver suggest a thromboembolism occluding the ductus venosus or other veins. superior vena cava. and right atrium ii. Complete: abnormal connection of umbilical vein (venous shunt) into iliac vein. In such cases of portal-ductal occlusion. and the second includes fetuses in whom the umbilical vein drains into the portal vein.37. a fundamental embryologic maldevelopment. and some were associated with pleural effusion. prolonged hyperperfusion of fetal liver sinusoids may induce prenatal portal hypertension. hydrops. connecting to the systemic venous circulation as described above. Vitelline veins a. in experiments involving rats.22. Partial Reprinted (order modified) by permission from Ultrasound in Obstetrics and Gynecology. might cause this anomaly. In addition. Cardinal vein a. Primary failure to create critical anastomoses i. blood is then diverted through other channels. heterotaxic syndrome b.34 In the latter. Noteworthy is a common feature of all the aforementioned cases: agenesis of the ductus venosus. Complete agenesis of portal system ii.9.39 Although complete primary failure to form the critical anastomoses in both the left and right 1150 umbilical veins is very rare.10. Partial: persistent right umbilical vein with or without ductus venosus b. thus causing all the umbilical blood to pass through the hepatic sinusoids.16. Diagnosis of this entity is feasible today with the widespread use of prenatal ultrasonography. In the former group.22. in which partial anastomosis between the hepatic sinusoids and umbilical vein fails to develop.16 vena cava.46 J Ultrasound Med 21:1145–1158. or a direct communication with the right atrium or the left horn of the sinus venosus (coronary sinus. Total b. In these cases. inferior vena cava. In such cases. The umbilical vein drains properly into the portal vein but fails to establish a communication with the persistent proximal part of the right vitelline vein. the most common of which is the persistent right umbilical vein anomaly (Fig. primary or secondary to occlusion by thromboembolic events arising from the placenta. Anomalous pulmonary venous connection a. Primary failure to create critical anastomoses i. Jeanty9 suggested that streaming of early flow through the right umbilical vein.21. 2002 . several cases of neonates with this anomaly have since been reported. teratogenic agents such as retinoic acid and folate deficiency were found to induce primary failure to form anastomosis and consequently the right umbilical vein to remain patent. Isolated malformation B. or to the inferior vena cava in its infracardiac segment. 3). Secondary occlusion C.43. possibly a secondary formation of such an anomaly.15. Although agenesis of the ductus venosus was first described more than 100 years ago.42 whereas the second.
Fasouliotis et al Anomalies of the Vitelline Veins Abnormalities of the vitelline veins are extremely rare. Complete absence of the portal system presents an extreme example of total failure of the vitelline veins to transform into the portal system. the enterohepatic circulation is disturbed. As a result. and marked dilatation of the inferior vena cava. or directly into the inferior vena cava. and only a few have been reported during fetal life. A B the portal system or partial agenesis of the right or left portal branch. IVC. inferior vena cava. C J Ultrasound Med 21:1145–1158. Mesenteric and splenic venous blood may drain into the renal veins. GB. with a persistent left vitelline vein connected directly to the hepatic vein (portohepatic shunt) and an absence of the ductus venosus. hepatic veins.50 Incomplete absence of the portal system or partial failure to form critical anastomosis may represent a more benign form of vitelline vein abnormalities.47–49 To our knowledge. and asterisk. AO indicates aorta. 2002 1151 . with neonatal spontaneous resolution Figure 4. and dotted arrow. The diagnosis was confirmed postnatally. B. coronary sinus.16 Primary failure to form the critical anastomosis may lead to complete agenesis of Figure 3. and the portal venous blood is shunted systemically. gallbladder. and UV. ultrasonographic prenatal diagnosis of congenital absence of the portal system is rare and has only been described in a single case in which the in utero ultrasonographic appearance of total agenesis of the portal system included an intrahepatic aberrant vessel. Persistent right umbilical vein anomaly. The arrow indicates the dilated umbilical vein.13. C. Fetal echocardiography revealed cardiomegaly with dextrocardia and anomalous venous return to the coronary sinus with agenesis of the ductus venosus. stomach. absence of the portal system. stemming from a primary failure to form critical anastomosis with hepatic sinusoids or umbilical veins. Partial primary failure to form critical anastomosis may result in agenesis of the right portal system. Images from a patient referred at 22 gestational weeks for investigation of cardiomegaly. umbilical vein. The solid arrow indicates an aberrant vessel. A. The arrow indicates the right portal vein.
Common forms of PAPVC. Anomalous connection of the right pulmonary veins (R.V. Usually these malformations are also accompanied by an atrial septal defect.S. left ventricle. 5). A. to our knowledge.). Anomalous connection of the left pulmonary veins (L. Children.A.51 In addition.). B. Anomalous connections of the right pulmonary veins to the inferior vena cava (I.) by way of a vertical vein (V. C. A high or sinus venous defect is usual in this anomaly.. only 1 case with agenesis of the right and left portal veins associated with a portosystemic shunt has been reported prenatally. of the pulmonary veins connecting to the right atrium or a tributary (Fig.P.16.V.V.). The right lung commonly drains by 1 pulmonary vein without its usual anatomic divisions.) to the superior vena cava (S.. indicates left atrium. right ventricle. and the atrial septum is usually intact. D.V. R. L.V. right atrium. 2002 .V. Parenchymal abnormalities of the right lung are common. L. Reprinted by permission from Heart Disease in Infants. An additional left-to-right shunt may occur through the atrial septal defect.V. Anomalous connection of the left pulmonary veins to the coronary sinus (C.1 1152 J Ultrasound Med 21:1145–1158.Inn. Partial anomalous pulmonary venous connection (PAPVC) involves 1 or more. and Adolescents.A.The Human Fetal Venous System occurring later in some of these cases.). and R. Figure 5.C.C.) to the left innominate vein (L.V. but not all.P.17 Anomalous Pulmonary Venous Connection Anomalous pulmonary venous connection may be separated into 2 subgroups: partial and total. Several variant manifestations of this anomaly have been reported and include abnormal insertions of the right pulmonary veins into the superior or inferior vena cava and the right atrium and entry of the left pulmonary veins into the left innominate vein.
An atrial septal defect or patent foramen ovale is generally present. The presence of an obstructive lesion in the pulmonary venous channel influences the hemodynamic state and clinical manifestation of TAPVC. TAPVC is classified into 4 types. This obstruction may occur at the interatrial septum or may be intrinsic or extrinsic to the anomalous venous channel. 2002 The aim of echocardiographic evaluation of suspected TAPVC lies in locating the site of the connection of the common pulmonary vein. maintaining ductal patency by using prostaglandins is of little help. In infradiaphragmatic forms of TAPVC. the subset of patients with an anomalous connection to the coronary sinus does not tend to have urgent symptoms. and confirming or excluding associated cardiac lesions. Doppler studies reveal flow in this vertical vessel to be cephalad. it is one of the few emergencies left in pediatric cardiologic practice. pulmonary atresia. and the presence of any cardiac or extracardiac malformations. An alternate classification system divides this anomaly into 2 groups: supradiaphragmatic without pulmonary venous obstruction and infradiaphragmatic with pulmonary venous obstruction. as opposed to the caudal flow found in the superior vena cava. In addition. an enlarged right atrium. 6). or it may appear as a discrete chamber. depending on the level of the anomalous connection. and unlike other forms of congenital heart disease. On the other hand.53 All forms of TAPVC will have right ventricle volume overload. or 2 or more of the above levels (IV). the overall state of the pulmonary vascular bed. In one system. with the pulmonary veins draining into it. The prognosis in cases of PAPVC and TAPVC depends on the presence and size of the interatrial connection. truncus arteriosus. infracardiac level (III). About one third of patients have an associated major anomaly such as cor biloculare. and a bowing leftward of the interatrial septum. occurring in only about 1 per 17. or coronary sinus. evaluating any obstruction of the pulmonary veins or vertical vein. Also. the common pulmonary channel may be identified posterior to the left atrium. coarctation. most commonly the left innominate vein or superior vena cava.Fasouliotis et al Visualization of pulmonary veins connecting to the right-sided cardiac structure involved. whereas pulsed or continuous wave Doppler imaging allows for quantification of the degree of obstruction. Doppler flow studies and color mapping are indispensable in identification and classification of this group of anomalies. thus the pulmonary veins drain into the right atrium or the systemic veins (Fig.52 Several classification systems for TAPVC have been proposed. hypoplastic left ventricle. single ventricle. which in turn may be followed to a dilated systemic venous structure. If untreated. a finding of right ventricular volume overload with an intact atrial septum may mean that a PAPVC anomaly is present. whether the anomalous connection is at the supracardiac level (I). the presence of any obstructive lesion in the anomalous venous pathways. This is because neonates with obstructed pulmonary venous return can have severe decompensation soon after birth. is the basis of echocardiographic diagnosis of PAPVC. small and inferior to the left atrium above the diaphragm. affected neonates will survive only a few days to approximately 4 months. Doppler examination is invaluable in the diagnosis of all forms of TAPVC. cardiac level (II). J Ultrasound Med 21:1145–1158. In total anomalous pulmonary venous connection (TAPVC) there is no connection between the pulmonary veins and the left atrium. the neonate usually dies in the first weeks of life. left innominate vein.54–59 1153 . Total anomalous pulmonary venous connection should be suspected when no pulmonary veins can be visualized entering the left atrium on the short-axis scan. the channel will usually drain into an ascending vertical vein. and anomalies of the systemic veins. In supracardiac forms of TAPVC. Turbulence from any obstruction will produce a mosaic color jet that reveals its location. The abnormal color flow signal may be followed to identify the anomalous connection as well as the direction and mean velocity of flow. The channel may be followed to the site of its connection. whereas the remaining two thirds have isolated TAPVC. Although TAPVC is a rare lesion in its isolated form. because the drainage to this site is rarely obstructed. which may be the superior vena cava. The pulmonary veins converge into a common channel. In cases of TAPVC with obstruction in the anomalous venous channel. the connection is usually at the portal venous system but may also be to the hepatic veins. The most common anomalies of the fetal systemic venous system diagnosed prenatally that have been described recently are summarized in Table 3. transposition of the great arteries.000 live births.
. S. superior vena cava. S.V.P. left ventricle.P.A.1 1154 J Ultrasound Med 21:1145–1158. R.H.) by way of the ductus venosus (D.P. R..C. A.P. Children. D. and Adolescents.. Common forms of TAPVC.Inn.V.A.). This connects to the portal vein...).V.The Human Fetal Venous System Figure 6. right hepatic vein. B.).) usually enter the right atrium separately. TAPVC to the left innominate vein (L.V.V. indicates left atrium.) or the hepatic sinusoids.S. which connects to the coronary sinus.V..V. TAPVC to the portal vein (P.P. right portal vein.). L. and R.V.. L. right ventricle. 2002 . TAPVC to the coronary sinus (C. and S.V. L. C.. splenic vein. right atrium.. Reprinted by permission from Heart Disease in Infants. TAPVC to the right atrium.V.) by way of a vertical vein (V. The pulmonary veins join to form a confluence designated the common pulmonary vein (C.V.V. R. left hepatic vein. L.M. superior mesenteric vein. R. left portal vein. The pulmonary veins form a confluence from which an anomalous channel arises.H. which communicates with the inferior vena cava (I.V..C. The left and right pulmonary veins (L.
NA. SA 4. SIT. and TOP. neonatal death. TOP 10. 4. termination of pregnancy. SI. 1. complete atrioventricular septal defect 5. not available. dilated inferior vena cava. cardiomegaly. SIT 2 1. cardiomegaly. AV. dysrhythmias Congenital heart disease 10. PSS 1. Common Anomalies of the Venous System Anomaly No. RI 5. no portal vein Liver hyperechoic foci. PSS. asymmetry of cardiac chambers: right > left 2. IUGR 60. without additional findings. right isomerism. alive. of Cases Sonographic Findings/Associated Abnormalities Outcome Cardinal veins Right and left isomerism Atkinson and Drant54 13 8. neonatal death. 1. RI. 45. situs inversus. IUFD. A&W no ductus venosus. hydrops. 1. intrauterine fetal death. hydrops 2. 1. lost to follow-up 68. 2002 1155 . alive 2. 1. SIT NA NA NA 5 4 10 2. cardiomegaly. polysplenia syndrome. 1. dilated intraabdominal segment of umbilical vein A&W A&W 1 1 Portohepatic shunt. LI 7. TOP. drainage to the coronary sinus. drainage to the inferior vena cava. J Ultrasound Med 21:1145–1158. TOP. no portal vein. interrupted inferior vena cava. increased nuchal translucency. 1. alive after successful surgical repair A&W indicates alive and well. polyhydramnios. drainage to the right superior vena cava.X 3. 1. IUFD. right cardiomegaly. 1. 5. died after surgery Healthy after birth Persistent right umbilical vein Blazer et al58 69 Shen et al10 Vitelline veins Complete agenesis of portal system Laverdiere et al50 Venhat-Raman et al59 Partial agenesis of portal system Achiron et al16 8 1 1 Unusual C-shaped vessel between the umbilical vein and dilated inferior vena cava. SI 1. diaphragmatic hernia. healthy after birth 1. 1. liver hyperechoic foci. 4. 2. neonatal death 3.Fasouliotis et al Table 3. 3. left isomerism. 1. IUGR. A&W partial agenesis of portal system Umbilical right atrial shunt. A&W. 1. delivered with all associated abnormalities Heterotaxy syndromes Marton et al55 Cesko et al56 Oztunc et al57 Umbilical veins Agenesis of ductus venosus Hofstaetter et al13 Achiron et al16 Contratti et al38 13 9. atrioventricular. 4. 1. asymmetry of cardiac chambers: right > left 4. asplenia syndrome. 1. IUFD 1. LI. transient nuchal findings. oligohydramnios. situs inversus totalis. neonatal death. 3. 7. AV canal 10. intrauterine growth restriction. without additional findings. abnormalities of viscerocardiac status 7. hydrops. 2. dextrocardia and right-sided descending aorta 4. 4. great vessel anomaly None Transposition of the great vessels Cardiovascular malformation Transposition of the great vessels 3. partial agenesis of the portal system Pulmonary Veins TAPVCs Achiron et al16 Allan and Sharland53 4 4 2. chromosomal abnormality. 2. minor anomalies. 5.
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