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CASE SERIES
Aberrant Drainage of the Umbilical Vein Into the Coronary Sinus Without Ductus Venosus Agenesis Foued Ben Brahim, MD, Tristan Hazelzet, MD, Laurence Cohen, MD, Isabelle Durand, MD, Julie Blanc, MD, Elise Barre, MD, Marie Brasseur Daudruy, MD, Nadine David, MD 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. Key Words—coronary sinus; ductus venosus; fetal echocardiography; fetus; obstetric ultrasound
C Received April 24, 2013, from the Department of Obstetrics and Gynecology, Sud Francilien Hospital, Corbeil-Essonnes, France (F.B.B.); Departments of Congenital Cardiology (T.H., I.D., E.B., N.D.) and Pediatric and Fetal Radiology (M.B.D., N.D.), Rouen University Hospital, Rouen, France; and Congenital Cardiology, Massy, France (L.C., J.B.). Revision requested June 3, 2013. Revised manuscript accepted for publication June 30, 2013. We thank Reuven Achiron, MD, for valuable advice on manuscript preparation. Address correspondence to Foued Ben Brahim, MD, Department of Obstetrics and Gynecology, Sud Francilien Hospital, 116 rue Jean Jaures, F- 91100 Corbeil-Essonnes, France. E-mail: [email protected]. doi:10.7863/ultra.33.3.535
ongenital abnormalities of the venous system in fetuses have been described in a number of studies.1–7 Ectopic connections of the umbilical vein have been described in cases of ductus venosus agenesis in particular. In extrahepatic forms, the ectopic connections of the umbilical vein most frequently reported are those to the iliac vein, the inferior vena cava, direct connections to the right atrium, and, more rarely, to the coronary sinus. In intrahepatic forms, the umbilicoportosystemic shunt connects the umbilical vein to the right portal vein.8,9 In cases in which the ductus venosus is present, only its abnormally low connection to the hepatic portion of the inferior vena cava has been described.10 We report 4 cases of abnormal connections of the ductus venosus to the coronary sinus detected prospectively between August 2011 and February 2012 in 2 congenital cardiologic centers.
Case Descriptions Case 1 A pregnant woman was seen in August 2011, at 32 weeks’ menstrual age, by a cardiologist at a pediatric cardiology center for an atrial abnormality. Fetal echocardiography (Vivid 7 Pro; GE Healthcare, Milwaukee, WI) showed a dilated coronary sinus as an explanation for the atrial abnormality (Figure 1). No left superior vena cava was visible on the 4-chamber or 3-vessel views, and the pulmonary veins seemed to empty normally into the left atrium. The ductus venosus seemed to drain into the coronary sinus. Doppler flux in the
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umbilical vein was monophasic, becoming triphasic, as normal, at the source of the ductus venosus, eliminating the possibility of ductus agenesis. The ductus venosus was normal in appearance and had normal triphasic flow, but it followed an abnormal direction, running parallel to the inferior vena cava and emptying into the dilated coronary sinus. Color Doppler imaging showed the coronary sinus emptying into the right atrium by a large ostium. As this malformation had not been reported before, the patient was referred for delivery to a maternity unit with easy access to a cardiac surgery unit, in case the child presented with a mistaken totally anomalous pulmonary vein connection. A girl weighing 3000 g was born at term, with no particular problems. Clinical examination of the neonate
showed normal findings. Postnatal echocardiography, done at 2 days of life, confirmed substantial dilatation of the coronary sinus, with a large ostium, no left superior vena cava, and no abnormality of the pulmonary veins. No abnormal vessels were detected. The child was doing well at the age of 8 months. Cases 2–4 Three patients were referred for fetal echocardiography between December 2011 and February 2012, at 20 to 22 weeks’ gestation, at a second pediatric cardiology unit. Two were referred for atrial abnormalities and 1 for coronary sinus dilatation. The sonographic data (α10; Aloka Co, Ltd, Tokyo, Japan) for the 3 fetuses were identical: the
A
Figure 1. Case 1. A, Four-chamber view in a slightly caudal plane showing dilatation of the coronary sinus. B, Subcostal view showing nonconfluence of the inferior vena cava and ductus venosus. CS indicates coronary sinus; DV, ductus venosus; IVC, inferior vena cava; LV, left ventricle; RA, right atrium; RV, right ventricle; and UV, umbilical vein.
B
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atrial abnormality was attributed to coronary sinus dilatation (Figures 2–4). The 4-chamber view was normal. The heart had a normal volume and normal morphologic characteristics. No left superior vena cava was visible on the 3-vessel view, and the pulmonary veins emptied normally into the left atrium. Abnormal high-speed triphasic flow closely resembling that of the ductus venosus was recorded in the coronary sinus in each fetus. The umbilical vein followed a normal trajectory (left umbilical vein), with normal
monophasic flux. The ductus venosus was normal in appearance and had normal triphasic flow, but it followed an abnormal direction, running parallel to the inferior vena cava. It emptied into the coronary sinus. The hepatic and portal veins were normal in appearance. Three-dimensional sonography (GE high-definition flow imaging) was performed in these 3 cases and confirmed the lack of confluence between the ductus venosus and the inferior vena cava and drainage of the ductus venosus into the dilated coronary sinus.
Figure 2. Case 2. A and B, Four-chamber views in a slightly caudal plane showing dilatation of the coronary sinus and arrival of rapid blood flow in the coronary sinus and right atrium by a large ostium (e-flow imaging). C, High-definition flow image showing nonconfluence of the inferior vena cava and ductus venosus. D, Sagittal view showing a normal venous system and normal confluence of the ductus venosus with the inferior vena cava (eflow imaging). dA indicates descending aorta; and SHV, sus-hepatic vein; other abbreviations are as in Figure 1. A
B
C
D
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All infants had no neonatal distress and were doing well. Postnatal echocardiography in the first week of life confirmed dilatation and permeability of the coronary sinus without any cardiac malformation. Abdominal sonography showed normal systemic veins and normal portal vessels.
Discussion The coronary sinus of the fetus is visible on a posterior section of the 4-chamber view. It is usually less than 3 mm in diameter.11,12 Its dilatation results in an intra-atrial image that may be mistaken for a persistent ostium primum atrial
Figure 3. Case 3. A, Four-chamber view in a slightly caudal plane showing dilatation of the coronary sinus. B, E-flow image showing nonconfluence of the ductus venosus with the inferior vena cava. C, Doppler pulsed flow image at the ostium of the coronary sinus. PV indicates portal vein; other abbreviations are as in Figures 1 and 2. A
B
C
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septal defect.13 In 3 of our observations, attention focused on the abnormal appearance of the atrium with, in 1 case, the impression of a septal defect of the persistent ostium primum type. In 1 case, dilatation of the coronary sinus was diagnosed. In this case, an abnormally large distance between the descending aorta and the left atrium led to suspicion of a totally anomalous pulmonary vein connection.14
Coronary sinus dilatation is often secondary to a connection to a persistent left superior vena cava.15 This diagnosis is easy to confirm by direct visualization of the left superior vena cava on the 4-chamber and 3-vessel views. If there is no left superior vena cava, coronary sinus dilatation should lead physicians to search for a totally anomalous pulmonary vein connection.16 In our 4 cases, normal
Figure 4. Case 4. A, Large distance between the descending aorta and left atrium. B, Dilatation of the coronary sinus and its ostium. C and D, Sagittal views showing dilatation of the coronary sinus and abnormal connection of the ductus venosus (contined). A
B
C
D
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pulmonary vein connections were shown by color Doppler scans in each fetus. The ductus venosus was normal in appearance and had normal triphasic flow, but it followed an abnormal direction, running parallel to the inferior vena cava and emptying into the dilated coronary sinus. Color Doppler imaging showed the coronary sinus emptying into the right atrium by a large ostium, eliminating atresia of the ostium of the coronary sinus. Exceptionally, in cases of ductus venosus agenesis, the umbilical vein may drain into the coronary sinus, in most cases after following an ectopic extracardiac trajectory. Ectopic ductus venosus connections are rare, and only forms in which the confluence of the ductus venosus with the inferior vena cava is abnormally low have been described. A large proportion of such cases are patients with Down syndrome.10 The umbilical vein followed a normal trajectory in our 4 cases (left umbilical vein). This vein then continued normally, in the form of a ductus venosus of normal appearance, as shown by the normal Doppler scans. The portal system was also normal in appearance. Only the distal portion of the ductus venosus followed an abnormal trajectory, running parallel to the inferior vena cava, into which the hepatic veins drained normally. To our knowledge, this anatomic form of ectopic umbilical vein drainage has not previously been reported. It may correspond to the connection, in the embryo, of the
ductus venosus to the proximal part of the left vitelline vein (left hepatic common cardinal vein), rather than in the right, as would normally be the case (Figure 5).17,18 In this venous abnormality, the distribution of umbilical venous blood flow is unaffected, and the vascularization of the portal system is normal. There is no umbilicosystemic shunt and, therefore, no risk of fetal heart failure. Blood from the ductus venosus arrives in the right atrium normally but with an abnormal orientation that does not facilitate shunting to the foramen ovale.19 The oxygenated blood of the ductus venosus may be mixed with systemic venous blood, possibly resulting in a marked decrease in oxygen saturation in the left part of the heart. At birth, the umbilical cord section causes spontaneous closure of the umbilical vein. The only image that persists on postnatal echocardiography is a dilated coronary sinus. It seems likely that the scarcity of information concerning this malformation is because it has no consequences, either before or after the birth. Furthermore, imaging of the coronary sinus is not always recommended for the screening of congenital cardiac malformations. The persistence of a left superior vena cava is often diagnosed because it is visible on the 4-chamber and 3-vessel views, which are systematically obtained. By contrast, coronary sinus dilatation is less often detected because this abnormality is seen from a posterior section of the 4-chamber or long-axis view, which is not systematically used in cardiac
Figure 4. (contined) E and F, E-flow images showing nonconfluence of the ductus venosus and inferior vena cava. AA indicates ascending aorta; and LA, left atrium; other abbreviations are as in Figures 1 and 2. E
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fetal screening. During fetal development, searches for associated abnormalities are important in all cases of venous abnormalities to exclude a genetic or polymalformation syndrome.10–22 In our series, no associated cardiac or extracardiac lesion was noted except a single umbilical artery. In conclusion, on the basis of the discovery of abnormal coronary sinus dilatation on sonography in 4 fetuses, we are able to describe an unknown abnormal umbilical vein connection. This variant should be considered in cases of isolated coronary sinus dilatation after exclusion of the most frequent cause (the presence of a left superior vena cava) and the most serious cause (a totally anomalous pulmonary vein connection). Unlike the rare cases of direct Figure 5. Embryologic hypothesis: ductus venosus drainage in a persistent left hepatic common cardinal vein. ALCV indicates anterior left cardinal vein; ARCV, anterior right cardinal vein; AzV, azygos vein; HCC, right and left hepatic common cardinal veins; LSCV, left supracardinal vein; LVV, left vitelline vein; LUV, left umbilical vein; PLCV, posterior left cardinal vein; PRCV, posterior right cardinal vein; RSCV, right supracardinal vein; RVV, right vitelline vein; RUV, right umbilical vein; SV, sinus venosus; and SVC, superior vena cava; other abbreviations are as in Figures 1 and 3. Modified from Yagel et al17 and Langman.18
connections of the umbilical vein in the absence of a ductus venosus, this abnormality has no consequences for fetal hemodynamics, undoubtedly accounting for the fact that it has long remained unrecognized. This condition does not require any particular management during the neonatal period if no associated abnormalities are found.
References 1.
2.
3. 4.
5.
6.
7.
8.
9.
10.
11.
12. 13.
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Yagel S, Kivilevitch Z, Cohen SM, et al. The fetal venous system, part II: ultrasound evaluation of the fetus with congenital venous system malformation or developing circulatory compromise. Ultrasound Obstet Gynecol 2010; 36:93–111. Achiron R, Gindes L, Kivilevitch Z, et al. Prenatal diagnosis of congenital agenesis of the fetal portal venous system. Ultrasound Obstet Gynecol 2009; 34:643–652. Hofstaetter C, Plath H, Hansmann M. Prenatal diagnosis of abnormalities of the fetal venous system. Ultrasound Obstet Gynecol 2000; 15:231–241. Viora E, Sciarrone A, Bastonero S, et al. Anomalies of the fetal venous system: a report of 26 cases and review of the literature. Fetal Diagn Ther 2004; 19:440–447. Achiron R, Hegesh J, Yagel S, Lipitz S, Cohen SB, Rotstein Z. Abnormalities of the fetal central veins and umbilico-portal system: prenatal ultrasonographic diagnosis and proposed classification. Ultrasound Obstet Gynecol 2000; 16:539–548. Kivilevitch Z, Gindes L, Deutsch H, Achiron R. In-utero evaluation of the fetal umbilical-portal venous system: two- and three-dimensional ultrasonic study. Ultrasound Obstet Gynecol. 2009; 34:634–642. Barrea C, Ovaert C, Moniotte S, Biard JM, Steenhaut P, Bernard P. Prenatal diagnosis of abnormal cardinal systemic venous return without other heart defects: a case series. Prenat Diagn 2011; 31:380–388. Acherman RJ, Evans WN, Galindo A, et al. Diagnosis of absent ductus venosus in a population referred for fetal echocardiography: association with a persistent portosystemic shunt requiring postnatal device occlusion. J Ultrasound Med 2007; 26:1077–1082. Gorincour G, Droulle P, Guibaud L. Prenatal diagnosis of umbilicoportosystemic shunts: report of 11 cases and review of the literature. AJR Am J Roentgenol 2005; 184:163–168. Achiron R, Gindes L, Gilboa Y, Weissmann-Brenner A, Berkenstadt M. Umbilical vein anomaly in fetuses with Down syndrome. Ultrasound Obstet Gynecol 2010; 35:297–301. Chaoui R, Heling KS, Kalache KD. Caliber of the coronary sinus in fetuses with cardiac defects with and without left persistent superior vena cava and in growth-restricted fetuses with heart-sparing effect. Prenat Diagn 2003; 23:552–557. Rein AJ, Nir A, Nadjari M. The coronary sinus in the fetus. Ultrasound Obstet Gynecol 2000; 15:468–472. Park JK, Taylor DK, Skeels M, Towner DR. Dilated coronary sinus in the fetus: misinterpretation as an atrioventricular canal defect. Ultrasound Obstet Gynecol 1997; 10:126–129. 541
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14. Valsangiacomo ER, Hornberger LK, Barrea C, Smallhorn JF, Yoo SJ. Partial and total anomalous pulmonary venous connection in the fetus: two-dimensional and Doppler echocardiographic findings. Ultrasound Obstet Gynecol 2003; 22:257–263. 15. Machevin-Surugue E, David N, Verspyck E, et al. Dilated coronary sinus in prenatal echocardiography: identification, associations and outcome. Prenat Diagn 2002; 22:898–902. 16. Karl K, Kainer F, Knabl J, Chaoui R. Prenatal diagnosis of total anomalous pulmonary venous connection into the coronary sinus. Ultrasound Obstet Gynecol 2011; 38:729–731. 17. Yagel S, Kivilevitch Z, Cohen SM, et al. The fetal venous system, part I: normal embryology, anatomy, hemodynamics, ultrasound evaluation and Doppler investigation. Ultrasound Obstet Gynecol 2010; 35:741–750. 18. Langman J. Abrégé d’Embryologie Médicale: Développement Humain, Normal et Pathologique. Paris, France: Masson; 1972. 19. Kiserud T, Eik-Nes SH, Blaas HG, Hellevik LR. Foramen ovale: an ultrasonographic study of its relation to the inferior vena cava, ductus venosus and hepatic veins. Ultrasound Obstet Gynecol 1992; 2:389-396. 20. Volpe P, Marasini M, Caruso G, et al. Prenatal diagnosis of ductus venosus agenesis and its association with cytogenetic/congenital anomalies. Prenat Diagn 2002; 22:995–1000. 21. Wolman I, Gull I, Fait G, et al. Persistent right umbilical vein: incidence and significance. Ultrasound Obstet Gynecol 2002; 19:562–564. 22. Pipitone S, Garofalo C, Corsello G, et al. Abnormalities of the umbilicoportal venous system in Down syndrome: a report of two new patients. Am J Med Genet A 2003; 120A:528–532.
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CASE SERIES
Aberrant Drainage of the Umbilical Vein Into the Coronary Sinus Without Ductus Venosus Agenesis Foued Ben Brahim, MD, Tristan Hazelzet, MD, Laurence Cohen, MD, Isabelle Durand, MD, Julie Blanc, MD, Elise Barre, MD, Marie Brasseur Daudruy, MD, Nadine David, MD 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. Key Words—coronary sinus; ductus venosus; fetal echocardiography; fetus; obstetric ultrasound
C Received April 24, 2013, from the Department of Obstetrics and Gynecology, Sud Francilien Hospital, Corbeil-Essonnes, France (F.B.B.); Departments of Congenital Cardiology (T.H., I.D., E.B., N.D.) and Pediatric and Fetal Radiology (M.B.D., N.D.), Rouen University Hospital, Rouen, France; and Congenital Cardiology, Massy, France (L.C., J.B.). Revision requested June 3, 2013. Revised manuscript accepted for publication June 30, 2013. We thank Reuven Achiron, MD, for valuable advice on manuscript preparation. Address correspondence to Foued Ben Brahim, MD, Department of Obstetrics and Gynecology, Sud Francilien Hospital, 116 rue Jean Jaures, F- 91100 Corbeil-Essonnes, France. E-mail: [email protected]. doi:10.7863/ultra.33.3.535
ongenital abnormalities of the venous system in fetuses have been described in a number of studies.1–7 Ectopic connections of the umbilical vein have been described in cases of ductus venosus agenesis in particular. In extrahepatic forms, the ectopic connections of the umbilical vein most frequently reported are those to the iliac vein, the inferior vena cava, direct connections to the right atrium, and, more rarely, to the coronary sinus. In intrahepatic forms, the umbilicoportosystemic shunt connects the umbilical vein to the right portal vein.8,9 In cases in which the ductus venosus is present, only its abnormally low connection to the hepatic portion of the inferior vena cava has been described.10 We report 4 cases of abnormal connections of the ductus venosus to the coronary sinus detected prospectively between August 2011 and February 2012 in 2 congenital cardiologic centers.
Case Descriptions Case 1 A pregnant woman was seen in August 2011, at 32 weeks’ menstrual age, by a cardiologist at a pediatric cardiology center for an atrial abnormality. Fetal echocardiography (Vivid 7 Pro; GE Healthcare, Milwaukee, WI) showed a dilated coronary sinus as an explanation for the atrial abnormality (Figure 1). No left superior vena cava was visible on the 4-chamber or 3-vessel views, and the pulmonary veins seemed to empty normally into the left atrium. The ductus venosus seemed to drain into the coronary sinus. Doppler flux in the
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umbilical vein was monophasic, becoming triphasic, as normal, at the source of the ductus venosus, eliminating the possibility of ductus agenesis. The ductus venosus was normal in appearance and had normal triphasic flow, but it followed an abnormal direction, running parallel to the inferior vena cava and emptying into the dilated coronary sinus. Color Doppler imaging showed the coronary sinus emptying into the right atrium by a large ostium. As this malformation had not been reported before, the patient was referred for delivery to a maternity unit with easy access to a cardiac surgery unit, in case the child presented with a mistaken totally anomalous pulmonary vein connection. A girl weighing 3000 g was born at term, with no particular problems. Clinical examination of the neonate
showed normal findings. Postnatal echocardiography, done at 2 days of life, confirmed substantial dilatation of the coronary sinus, with a large ostium, no left superior vena cava, and no abnormality of the pulmonary veins. No abnormal vessels were detected. The child was doing well at the age of 8 months. Cases 2–4 Three patients were referred for fetal echocardiography between December 2011 and February 2012, at 20 to 22 weeks’ gestation, at a second pediatric cardiology unit. Two were referred for atrial abnormalities and 1 for coronary sinus dilatation. The sonographic data (α10; Aloka Co, Ltd, Tokyo, Japan) for the 3 fetuses were identical: the
A
Figure 1. Case 1. A, Four-chamber view in a slightly caudal plane showing dilatation of the coronary sinus. B, Subcostal view showing nonconfluence of the inferior vena cava and ductus venosus. CS indicates coronary sinus; DV, ductus venosus; IVC, inferior vena cava; LV, left ventricle; RA, right atrium; RV, right ventricle; and UV, umbilical vein.
B
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atrial abnormality was attributed to coronary sinus dilatation (Figures 2–4). The 4-chamber view was normal. The heart had a normal volume and normal morphologic characteristics. No left superior vena cava was visible on the 3-vessel view, and the pulmonary veins emptied normally into the left atrium. Abnormal high-speed triphasic flow closely resembling that of the ductus venosus was recorded in the coronary sinus in each fetus. The umbilical vein followed a normal trajectory (left umbilical vein), with normal
monophasic flux. The ductus venosus was normal in appearance and had normal triphasic flow, but it followed an abnormal direction, running parallel to the inferior vena cava. It emptied into the coronary sinus. The hepatic and portal veins were normal in appearance. Three-dimensional sonography (GE high-definition flow imaging) was performed in these 3 cases and confirmed the lack of confluence between the ductus venosus and the inferior vena cava and drainage of the ductus venosus into the dilated coronary sinus.
Figure 2. Case 2. A and B, Four-chamber views in a slightly caudal plane showing dilatation of the coronary sinus and arrival of rapid blood flow in the coronary sinus and right atrium by a large ostium (e-flow imaging). C, High-definition flow image showing nonconfluence of the inferior vena cava and ductus venosus. D, Sagittal view showing a normal venous system and normal confluence of the ductus venosus with the inferior vena cava (eflow imaging). dA indicates descending aorta; and SHV, sus-hepatic vein; other abbreviations are as in Figure 1. A
B
C
D
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All infants had no neonatal distress and were doing well. Postnatal echocardiography in the first week of life confirmed dilatation and permeability of the coronary sinus without any cardiac malformation. Abdominal sonography showed normal systemic veins and normal portal vessels.
Discussion The coronary sinus of the fetus is visible on a posterior section of the 4-chamber view. It is usually less than 3 mm in diameter.11,12 Its dilatation results in an intra-atrial image that may be mistaken for a persistent ostium primum atrial
Figure 3. Case 3. A, Four-chamber view in a slightly caudal plane showing dilatation of the coronary sinus. B, E-flow image showing nonconfluence of the ductus venosus with the inferior vena cava. C, Doppler pulsed flow image at the ostium of the coronary sinus. PV indicates portal vein; other abbreviations are as in Figures 1 and 2. A
B
C
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septal defect.13 In 3 of our observations, attention focused on the abnormal appearance of the atrium with, in 1 case, the impression of a septal defect of the persistent ostium primum type. In 1 case, dilatation of the coronary sinus was diagnosed. In this case, an abnormally large distance between the descending aorta and the left atrium led to suspicion of a totally anomalous pulmonary vein connection.14
Coronary sinus dilatation is often secondary to a connection to a persistent left superior vena cava.15 This diagnosis is easy to confirm by direct visualization of the left superior vena cava on the 4-chamber and 3-vessel views. If there is no left superior vena cava, coronary sinus dilatation should lead physicians to search for a totally anomalous pulmonary vein connection.16 In our 4 cases, normal
Figure 4. Case 4. A, Large distance between the descending aorta and left atrium. B, Dilatation of the coronary sinus and its ostium. C and D, Sagittal views showing dilatation of the coronary sinus and abnormal connection of the ductus venosus (contined). A
B
C
D
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pulmonary vein connections were shown by color Doppler scans in each fetus. The ductus venosus was normal in appearance and had normal triphasic flow, but it followed an abnormal direction, running parallel to the inferior vena cava and emptying into the dilated coronary sinus. Color Doppler imaging showed the coronary sinus emptying into the right atrium by a large ostium, eliminating atresia of the ostium of the coronary sinus. Exceptionally, in cases of ductus venosus agenesis, the umbilical vein may drain into the coronary sinus, in most cases after following an ectopic extracardiac trajectory. Ectopic ductus venosus connections are rare, and only forms in which the confluence of the ductus venosus with the inferior vena cava is abnormally low have been described. A large proportion of such cases are patients with Down syndrome.10 The umbilical vein followed a normal trajectory in our 4 cases (left umbilical vein). This vein then continued normally, in the form of a ductus venosus of normal appearance, as shown by the normal Doppler scans. The portal system was also normal in appearance. Only the distal portion of the ductus venosus followed an abnormal trajectory, running parallel to the inferior vena cava, into which the hepatic veins drained normally. To our knowledge, this anatomic form of ectopic umbilical vein drainage has not previously been reported. It may correspond to the connection, in the embryo, of the
ductus venosus to the proximal part of the left vitelline vein (left hepatic common cardinal vein), rather than in the right, as would normally be the case (Figure 5).17,18 In this venous abnormality, the distribution of umbilical venous blood flow is unaffected, and the vascularization of the portal system is normal. There is no umbilicosystemic shunt and, therefore, no risk of fetal heart failure. Blood from the ductus venosus arrives in the right atrium normally but with an abnormal orientation that does not facilitate shunting to the foramen ovale.19 The oxygenated blood of the ductus venosus may be mixed with systemic venous blood, possibly resulting in a marked decrease in oxygen saturation in the left part of the heart. At birth, the umbilical cord section causes spontaneous closure of the umbilical vein. The only image that persists on postnatal echocardiography is a dilated coronary sinus. It seems likely that the scarcity of information concerning this malformation is because it has no consequences, either before or after the birth. Furthermore, imaging of the coronary sinus is not always recommended for the screening of congenital cardiac malformations. The persistence of a left superior vena cava is often diagnosed because it is visible on the 4-chamber and 3-vessel views, which are systematically obtained. By contrast, coronary sinus dilatation is less often detected because this abnormality is seen from a posterior section of the 4-chamber or long-axis view, which is not systematically used in cardiac
Figure 4. (contined) E and F, E-flow images showing nonconfluence of the ductus venosus and inferior vena cava. AA indicates ascending aorta; and LA, left atrium; other abbreviations are as in Figures 1 and 2. E
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fetal screening. During fetal development, searches for associated abnormalities are important in all cases of venous abnormalities to exclude a genetic or polymalformation syndrome.10–22 In our series, no associated cardiac or extracardiac lesion was noted except a single umbilical artery. In conclusion, on the basis of the discovery of abnormal coronary sinus dilatation on sonography in 4 fetuses, we are able to describe an unknown abnormal umbilical vein connection. This variant should be considered in cases of isolated coronary sinus dilatation after exclusion of the most frequent cause (the presence of a left superior vena cava) and the most serious cause (a totally anomalous pulmonary vein connection). Unlike the rare cases of direct Figure 5. Embryologic hypothesis: ductus venosus drainage in a persistent left hepatic common cardinal vein. ALCV indicates anterior left cardinal vein; ARCV, anterior right cardinal vein; AzV, azygos vein; HCC, right and left hepatic common cardinal veins; LSCV, left supracardinal vein; LVV, left vitelline vein; LUV, left umbilical vein; PLCV, posterior left cardinal vein; PRCV, posterior right cardinal vein; RSCV, right supracardinal vein; RVV, right vitelline vein; RUV, right umbilical vein; SV, sinus venosus; and SVC, superior vena cava; other abbreviations are as in Figures 1 and 3. Modified from Yagel et al17 and Langman.18
connections of the umbilical vein in the absence of a ductus venosus, this abnormality has no consequences for fetal hemodynamics, undoubtedly accounting for the fact that it has long remained unrecognized. This condition does not require any particular management during the neonatal period if no associated abnormalities are found.
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