Systemic and Pulmonary Venous Anomalies

  • Theresa Ann TacyEmail author


Assessment of systemic and pulmonary venous anatomy represents a vital part of the echocardiographic evaluation of any patient with suspected congenital heart disease (CHD). Systemic and pulmonary venous anomalies can occur as known associations with both simple and complex forms of CHD, and their presence (or absence) can have important clinical implications. Moreover, knowledge of systemic and pulmonary venous return is necessary for the accurate classification of some of the more complex forms of CHD, and for understanding the underlying physiology in such patients. Anyone performing echocardiographic evaluation of possible systemic/pulmonary venous anomalies should have a comprehensive understanding of the normal anatomy of both systems, as well as the more common abnormalities involving each system and associated hemodynamic alterations. Transesophageal echocardiography (TEE) is well suited for evaluation of both systemic and pulmonary venous anomalies, due to the proximity of the esophagus and transducer to the sites of venous return and to the atrial chambers. This chapter presents a thorough discussion of congenital systemic and pulmonary venous anomalies and their evaluation by TEE.


Transesophageal echocardiography Pulmonary venous anomalies Total anomalous pulmonary venous connection Partial anomalous pulmonary venous connection Systemic venous anomalies Intraoperative monitoring 

Supplementary material

Video 6.1

Transesophageal echocardiographic image of the right atrium viewed in the midesophageal bicaval view. The superior vena cava is seen superiorly with a small amount of contrast, and the right pulmonary artery is seen in cross section as it courses behind the SVC. The broad-based right atrial appendage is seen anteriorly (MOV 1409 kb)

Video 6.2

Image of the left atrium in the midesophageal two chamber view. The left upper pulmonary vein is seen, and directly anterior to this is the entrance of the narrow based left atrial appendage into the left atrium (MOV 1153 kb)

Video 6.3

The hepatic veins can be seen entering the right atrium in a nearly coronal plane from a transgastric window (MOV 5110 kb)

Video 6.4

In this view the TEE imaging probe is advanced to a low esophageal position to display the lower esophageal inferior vena cava long axis view as the probe angle is adjusted to the sagittal (90°) plane. The inferior vena cava (IVC) is demonstrated and a hepatic vein is seen joining the IVC anteriorly and superiorly (MOV 3091 kb) (629 kb)
Video 6.5 In the midesophageal four chamber view the probe has been retroflexed within the esophagus to demonstrate the coronary sinus and its relation to the left atrium (MOV 628 kb) (773 kb)
Video 6.6 In this image at 0°, the probe has been withdrawn and rotated to the left (counterclockwise) from the midesophageal four chamber view to demonstrate the relationship of the descending aorta (DAO), left pulmonary vein (LPV), and left atrial appendage (LAA) in a posterior to anterior position (MOV 773 kb) (1.3 mb)
Video 6.7 In this image at 0°, the probe has been rotated rightward (clockwise) in the midesophageal four chamber view and advanced slightly within the esophagus to demonstrate the entrance of the right lower pulmonary vein to the left atrium (MOV 1369 kb) (1.4 mb)
Video 6.8 A view of the left pulmonary veins from the midesophageal position as they enter the left atrium using an imaging plane of approximately 125° (MOV 1391 kb) (738 kb)
Video 6.9 A view of the right pulmonary veins from the midesophageal position, with color flow mapping as they join the left atrium. This view can be obtained with rightward rotation of the probe, using an imaging plane of approximately 30°–50° (MOV 737 kb)
Video 6.10

Midesophageal two chamber view displaying the left atrium and left ventricle. A dilated coronary sinus is demonstrated in its short axis (MOV 2485 kb)

Video 6.11

View of the coronary sinus in its longitudinal plane. This receives a left superior vena cava as demonstrated by color flow mapping. This view can be obtained by initially obtaining a short axis view of the coronary sinus and rotating the transducer to 60°–80° (MOV 4702 kb) (548 kb)
Video 6.12 Midesophageal two chamber view demonstrating a coronary sinus atrial septal defect (MOV 548 kb) (430 kb)
Video 6.13 Midesophageal bicaval view demonstrates the commitment of a right superior vena cava to the left atrium. Color Doppler demonstrates flow from the superior vena cava entering both the left atrium (red) and right atrium (blue) (MOV 429 kb)
Video 6.14

Equivalent view as depicted in Video 6.4 in a patient with interrupted inferior vena cava with azygous continuation. In this view only the hepatic veins are seen (MOV 1501 kb)

Video 6.15

In this view the TEE probe is in the low retrocardiac position, and has been rotated posteriorly in the sagittal plane. Two vascular structures are seen coursing adjacent to each other. Color Doppler demonstrates pulsatile flow directed caudally (the descending aorta) anteriorly and a second posterior structure, which has venous flow traveling towards cranially (the azygous vein) (MOV 3224 kb)

Video 6.16

View of the azygous vein as it travels towards the superior vena cava. At 90°, the distal azygous vein can be seen coursing over the right pulmonary artery seen in its short axis. This is a modified view obtained at the level of the mid esophagus (MOV 1065 kb)

Video 6.17

From the midesophageal ascending aortic short axis view (angle 0°) a teardrop shape of the superior vena cava indicates an anomalous pulmonary venous connection, in this case of a right upper vein to the superior vena cava (MOV 864 kb) (451 kb)
Video 6.18 Deep transgastric view in the sagittal plane demonstrating by color Doppler turbulent flow from a right lower pulmonary vein as it drains into the posterior aspect of the inferior vena cava above the level of the diaphragm (MOV 450 kb)
Video 6.19

Transesophageal echocardiogram in an infant with hypoplastic left heart syndrome in the midesophageal four chamber view demonstrating the usual appearance for the septum primum initially however as one withdraws the probe within the esophagus, the septum is seen shifting to a more leftwards position (MOV 3564 kb)

Video 6.20

This view is oriented to identify the right pulmonary veins. Close relationship between pulmonary veins (red flow) and pulmonary arteries (blue flow) is evident here—the pulmonary artery being directly anterior and usually slightly superior to the pulmonary veins (MOV 1500 kb)

Video 6.21

The video represents a representative sweep in an infant with total anomalous pulmonary venous connection to the left innominate vein. The exam is initiated at the midesophageal four chamber view. From this window, modified views at the level of the mid esophagus display the right upper and lower pulmonary veins (RUPV, RLPV) and left pulmonary veins (LPVs) are seen returning to a large horizontal confluence. A large vertical vein (VV) arises from the left side of the confluence and travels anteriorly and superiorly, coursing over the left pulmonary artery (LPA) to insert into the left innominate vein (Inn V). The length of the vertical vein is best seen in a sagittal plane, with the midesophageal ascending aortic and upper esophageal aortic arch short axis views, and leftward rotation of the probe. There is no obstruction at any point to pulmonary venous return. A catheter placed in the left internal jugular vein is seen in the vertical vein by two-dimensional imaging. On this video, prominent electrocautery and Doppler mirror image artifacts are seen (MPG 38454 kb)

Video 6.22

In this midesophageal four chamber view a pulmonary venous confluence is shown adjacent to the left atrium (circular structure) (MOV 3395 kb)

Video 6.23

Orthogonal view from that shown in Video 6.22. Color Doppler interrogation of pulmonary venous confluence in infant with total anomalous pulmonary venous connection as displayed in the long axis. The blue flow away from the transducer suggests an infradiaphragmatic course (MOV 2111 kb)

Video 6.24

Midesophageal four chamber view with clockwise transducer rotation demonstrating direct drainage of a right lower pulmonary vein to the right atrium (MOV 1787 kb)

Video 6.25

In this midesophageal four chamber view a dilated coronary sinus is seen (MOV 3372 kb) (1.1 mb)
Video 6.26 This is an image specifically examining the right pulmonary veins after direct anastomosis of the pulmonary venous confluence to the left atrium. Torsion of the anastomosis resulted in localized narrowing of the right upper pulmonary vein (MOV 1157 kb)
Video 6.27

Corresponding image to that displayed in Video 6.26 with color flow Doppler across right upper pulmonary vein demonstrating narrowing resulting from torsion of the pulmonary venous confluence to the left atrial anastomosis (MOV 1017 kb) (1.1 mb)
Video 6.28 In this midesophageal four chamber view the anastomotic site between the pulmonary venous confluence and left atrium is well seen. This appears widely patent by two-dimensional imaging (MOV 1144 kb) (966 kb)
Video 6.29 Modified midesophageal bicaval view performed post repair of sinus venosus atrial septal defect. Flow disturbance was seen near the cardiac end of the superior vena cava suggestive of obstruction. This led to further investigation and revision of the repair (MOV 965 kb)


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© Springer-Verlag London 2014

Authors and Affiliations

  1. 1.Division of Pediatric Cardiology, Department of PediatricsLucile Packard Children’s Hospital, Stanford University School of MedicinePalo AltoUSA

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