Abnormalities of the Systemic and Pulmonary Veins

  • Marietta Charakida
  • John Simpson


Abnormalities of the systemic and pulmonary veins can occur in isolation or in conjunction with other congenital heart defects. When venous anomalies present in isolation it can be particularly difficult to detect them during routine fetal anomaly scans as these are relatively small extra-cardiac structures. However, advances in ultrasound technology with higher resolution on 2D and colour Doppler ultrasound have facilitated and improved prenatal detection. The systemic venous variations and abnormalities, which will be discussed in this chapter include persistent left superior vena cava, interrupted inferior vena cava and agenesis of the ductus venosus. Anomalies of the pulmonary veins including total or partial anomalous pulmonary venous connections may be difficult to diagnose in isolation. Disorders of laterality which can be associated with systemic and/or pulmonary venous abnormalities as well as intracardiac defects will also be addressed.


Left superior vena cava Anomalous pulmonary venous connection Interrupted inferior vena cava Isomerism Agenesis of the ductus venosus 

Supplementary material

Video 10.1

Transverse sweep from the fetal abdomen to the four-chamber view demonstrating the normal azygous vein. The small azygous vein is seen posterior and to the right of the descending aorta (MP4 8929 kb)

Video 10.2

Parasagittal view demonstrating the course of the umbilical vein towards the ductus venosus on 2D and colour. The usual course of the inferior vena cava toward the right atrium is also shown. At the origin of the DV, the blood coming from the umbilical vein accelerates leading to aliasing of the colour flow signal (MP4 3944 kb)

Video 10.3

Transverse view at the level of four chamber view demonstrating the dilated coronary sinus and pulmonary venous flow both on greyscale and colour flow Doppler. In cases where there is normal pulmonary venous drainage to the left atrium as demonstrated in this case, a dilated coronary sinus is suggestive of the presence of PLSVC (MP4 4042 kb)

Video 10.4

Transverse view at the level of three vessel view demonstrating the presence of four vessels. From right to left, the right superior vena cava, ascending aorta, main pulmonary artery/duct and left superior vena cava are seen. The full length of the main pulmonary artery and duct is seen with the other vessels in cross-section (MP4 926 kb)

Video 10.5

Transverse view at the level of the three vessel view demonstrating on colour the presence of single left superior vena cava to the left of the pulmonary artery. On sweeping cranially, right to left flow in the innominate vein drain to the left SVC can be visualised (MP4 3678 kb)

Video 10.6

Sagittal view of the fetal thorax and upper abdomen demonstrating the two parallel vessels of similar size “double vessel” appearance. The descending aorta is visualized in blue colour and the azygous vein in red. No inferior vena cava is seen passing through the liver. Interruption of the inferior vena cava with azygous continuation is strongly associated with left atrial isomerism (MP4 3693 kb)

Video 10.7

Sagittal view demonstrating direct drainage of the umbilical vein to the right atrium using colour flow Doppler. This fetus had agenesis of the ductus venosus. The normal inferior vena cava has a course from the abdomen to the right atrium. Parallel to the spine the normal descending aorta is visualized (blue colour) (MP4 17,923 kb)

Video 10.8

A transverse view at the level of four-chamber view demonstrating the smooth wall of the left atrium. No pulmonary veins are seen draining to the left atrium. A confluence is identified at the back of the left atrium suggestive of total anomalous pulmonary venous drainage (AVI 14809 kb)

Video 10.9A

Transverse sweep from the four-chamber view to the three vessel view demonstrating the presence of an ascending vein (red colour) at a place where a PLSVC is expected to be found (MP4 2362 kb)

Video 10.9B

In a more superior view the ascending vein is seen draining to the innominate vein (red flow) which joins the RSVC (MP4 2095 kb)

Video 10.10

Tranverse sweep from the four-chamber view to an inferior plane where the dilated coronary sinus is seen. The pulmonary veins can be seen in red joining the coronary sinus and draining to the right atrium (MP4 2232 kb)

Video 10.11

Fetus with infracardiac total anomalous pulmonary venous drainage. Longitudinal view of the fetal thorax and abdomen in colour demonstrating the descending aorta and a venous channel originating from the confluence of pulmonary veins and draining into the liver (MP4 5871 kb)

Video 10.12

Transverse view at the level of the three-vessel view of a fetus with left atrial isomerism. The left sided panel demonstrates dilated azygous vein draining into the SVC on 2D. This is confirmed on colour flow Doppler on the right sided panel image. The dilated azygous vein (red colour) draining into the superior vena cava is visualized. The aorta is seen in cross section and the pulmonary artery in blue colour (MP4 2882 kb)

Video 10.13

Transverse plane at the level of the four chamber view demonstrating the presence of two vessels of similar size “double vessel” in front of the spine (the azygous vein and the descending aorta) which suggests the presence of interrupted inferior vena cava and left atrial isomerism. The azygous vein is posterior and to the right. The colour Doppler flow demonstrates normal pulmonary venous drainage to the left atrium (MP4 6358 kb)


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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Marietta Charakida
    • 1
  • John Simpson
    • 1
  1. 1.Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Foundation TrustLondonUK

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