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Systemic and Pulmonary Venous Anomalies

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Transesophageal Echocardiography for Pediatric and Congenital Heart Disease

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Abstract

Assessing systemic and pulmonary venous anatomy is a vital component 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 accurately evaluating some of the more complex forms of CHD and for understanding the underlying physiology in such patients. Anyone evaluating possible systemic and pulmonary venous anomalies by echocardiography should have a comprehensive understanding of the normal anatomy of both systems, as well as the more common anomalies affecting each venous system and their associated hemodynamic alterations. Transesophageal echocardiography (TEE) is well suited for evaluating both systemic and pulmonary venous anatomy and anomalies thereof because of the proximity of the esophagus and imaging transducer to the sites of venous return and the atrial chambers. This chapter presents relevant aspects of systemic and pulmonary venous anatomy and a thorough discussion of congenital systemic and pulmonary venous anomalies, addressing their evaluation by TEE.

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Abbreviations

2D:

Two-dimensional

Ao:

Aortic

Asc:

Ascending

ASD:

Atrial septal defect

Atr:

Atrial

BCPA:

Bidirectional cavopulmonary anastomosis

CS:

Coronary sinus

Desc:

Descending

DTG:

Deep transgastric

Hep Veins:

Hepatic veins

IVC:

Inferior vena cava

LA:

Left atrium

LAA:

Left atrial appendage

LAX:

Long axis

Lf:

Left

LIV:

Left innominate vein

LLPV:

Left lower pulmonary vein

LPA:

Left pulmonary artery

LSVC:

Left superior vena cava

LUPV:

Left upper pulmonary vein

LV:

Left ventricle

ME:

Midesophageal

MRI:

Magnetic resonance imaging

PA:

Pulmonary artery

PAPVC:

Partial anomalous pulmonary venous connection

Pulm:

Pulmonary

RA:

Right atrium

RAA:

Right atrial appendage

RLPV:

Right lower pulmonary vein

RPA:

Right pulmonary artery

RSVC:

Right superior vena cava

Rt:

Right

RUPV:

Right upper pulmonary vein

RV:

Right ventricular

SAX:

Short axis

Sept:

Septal

SVC:

Superior vena cava

TAPVC:

Total anomalous pulmonary venous connection

TEE:

Transesophageal echocardiography

TG:

Transgastric

TTE:

Transthoracic echocardiography

UE:

Upper esophageal

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Authors and Affiliations

  1. Betty Irene Moore Children’s Heart Center, Lucile Packard Children’s Hospital, and , Stanford University School of Medicine, Palo Alto, CA, USA

    Theresa Ann Tacy & Shiraz Arif Maskatia

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  1. Theresa Ann Tacy
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  2. Shiraz Arif Maskatia
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Correspondence to Shiraz Arif Maskatia .

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Editors and Affiliations

  1. Division of Pediatric Cardiology, Department of Pediatrics, Children’s Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA

    Pierre C. Wong

  2. Department of Anesthesiology, Perioperative and Pain Medicine, Arthur S. Keats Division of Pediatric Cardiovascular Anesthesiology and Department of Pediatrics, Section of Cardiology, Baylor College of Medicine, Texas Children’s Hospital; Texas Heart Institute, Houston, TX, USA

    Wanda C. Miller-Hance

Electronic Supplementary Material

Image of the right atrial appendage (RAA) in a ME Bicaval view. The superior vena cava is seen to the right of the image display with a small amount of spontaneous contrast as it enters into the right atrium. The right pulmonary artery is displayed in cross-section (short axis) as it courses posterior to the SVC. The broad-based RAA is seen anteriorly (MP4 254 kb)

Image displaying the left atrial appendage (LAA) in a ME 2-Ch view. The left upper pulmonary vein is visible, and directly anterior to its entrance into the left atrium, the narrow-based LAA is seen. A mildly dilated coronary sinus is seen posteriorly along the atrioventricular groove (MP4 366 kb)

Transgastric view showing the hepatic veins entering the inferior vena cava in a nearly coronal plane. Please note that in this example the image has been up/down inverted from the standard transgastric image display. This view allows for optimal Doppler interrogation of hepatic venous flows (MP4 1009 kb)

TG IVC/Hep veins view obtained by advancing the imaging probe from the ME Bicaval view further into the lower esophagus/gastric region just beyond the level of the diaphragm. A hepatic vein is seen joining the inferior vena cava anteriorly and superiorly (MP4 399 kb)

Image obtained after advancing and retroflexing the imaging probe from the ME 4-Ch view showing the coronary sinus (CS) and its relation to the left atrium. Note the normal opening of the CS into the right atrium (MP4 424 kb)

ME Bicaval view after repair of a sinus venosus atrial septal defect. Color flow disturbance is seen near the cardiac end of the superior vena cava, suggesting obstruction. This led to further investigation and revision of the repair (MP4 783 kb)

ME 2-Ch view displaying a dilated coronary sinus in a patient undergoing mitral valve surgery (MP4 497 kb)

Image of a dilated coronary sinus (CS) in its longitudinal plane as it receives a left superior vena cava as shown by color flow mapping. This view can obtained after initially displaying the CS in short axis in the ME 4-Ch view and rotating the transducer angle forward to ~60°–80° (MP4 949 kb)

ME 2-Ch view of a coronary sinus atrial septal defect (MP4 431 kb)

ME Bicaval view showing commitment of a right superior vena cava to the left atrium. Color Doppler shows flow from the superior vena cava entering both the left atrium (larger red flow) and right atrium (smaller blue aliased flow) (MP4 368 kb)

TG IVC/Hep veins view in a patient with interrupted inferior vena cava with azygos continuation. Only the hepatic veins (not the IVC) are visible as they course through the liver. Refer to Video 6.4 for comparison (MP4 219 kb)

View in which the TEE probe is in a low esophageal retrocardiac position and has been rotated posteriorly to a sagittal plane. Two vascular structures are seen coursing adjacent to each other. Color Doppler shows pulsatile flow from the descending aorta directed caudally and a second structure, the azygos vein, which has venous flow coursing cranially (MP4 683 kb)

View of the azygos vein as it travels toward the superior vena cava (SVC). At a transducer angle of 90°, the distal azygos vein can be seen coursing over the right pulmonary artery, depicted in its short axis. Entry of the azygos vein into the SVC is not shown. This is a modified view obtained at the level of the upper esophagus (MP4 295 kb)

In this image at a transducer angle of 0°, the probe has been withdrawn and rotated to the left (counterclockwise) from the ME 4-Ch view to visualize—from posterior to anterior—the descending aorta (DAO), left upper pulmonary vein (LPV), and left atrial appendage (LAA) (MP4 610 kb)

ME Rt Pulm veins view obtained after the TEE probe was rotated rightward from the ME 4-Ch view to visualize the entrance of the right upper pulmonary vein into the left atrium (MP4 837 kb)

ME Lt Pulm veins view obtained as the probe shaft is turned leftward from the ME LAX view (transducer angle of 125°) to visualize both of the left-sided pulmonary veins (MP4 1020 kb)

View of the right pulmonary veins from the midesophageal position (transducer angle of 28°), with color flow mapping as they enter the left atrium. This view can be obtained with rightward rotation of the probe after the ME RV In-Out view has been obtained and the transducer angle adjusted to ~30°–50°. The right upper pulmonary vein is to the far right of the image and the right lower pulmonary vein to the far left (MP4 707 kb)

Image depicts a teardrop shape caused by an anomalous right upper pulmonary vein (RUPV) as it entered the superior vena cava (SVC). A change of the SVC from a round to a teardrop shape during real-time imaging is highly suggestive of an anomalous connection of the RUPV to the SVC. This view was obtained as the probe was rotated to the right from the ME Asc Ao SAX view (MP4 229 kb)

ME 4-Ch view with clockwise probe rotation after a Warden procedure, depicting the laminar flow across the rerouted anomalous right pulmonary vein by color Doppler (red flow) and the atrial septal patch. The repair incorporated the anomalous right pulmonary venous flow into the left atrium while patching the atrial septal defect (MP4 2666 kb)

Midesophageal view (transducer angle of 29°) displaying color Doppler interrogation of a pulmonary venous baffle after two-patch repair of a sinus venosus atrial septal defect and partial anomalous pulmonary venous connection (MP4 806 kb)

ME Bicaval view obtained from the same patient shown in Video 6.19, depicting flow across the superior vena cava into the right atrium (MP4 893 kb)

ME Bicaval view displaying a patch that incorporates the anomalous pulmonary venous return into the left atrium while separating the atria. Note the unobstructed pulmonary venous flow (MP4 832 kb)

ME Bicaval view showing flow (blue signal) across the anterior superior vena cava to right atrial appendage connection in the same patient depicted in Video 6.22 (MP4 6278 kb)

DTG Atr Sept view (transducer angle of 110°) depicting turbulent color flow from an anomalous right lower pulmonary vein or Scimitar vein, draining into the posterior aspect of the inferior vena cava just above the diaphragm (MP4 538 kb)

ME 4-Ch view in an infant with hypoplastic left heart syndrome. In the first few frames, the position of the septum primum appears normal. However, as the probe is withdrawn within the esophagus, the unusual left-shifted orientation of the septum primum becomes apparent, as seen in later frames (MP4 746 kb)

Midesophageal view (transducer angle of 72°) oriented to identify the right pulmonary veins. The close relationship between the pulmonary veins (red flow) and pulmonary arteries (blue flow) is evident in this image—the pulmonary artery being directly anterior and usually slightly superior to the pulmonary veins (MP4 291 kb)

Representative sweep in an infant with total anomalous pulmonary venous connection to the left innominate vein. The exam starts at the ME 4-Ch view. From this window, modified views at the level of the mid esophagus display the right upper and lower pulmonary veins (RUPV, RLPV), and the 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 the sagittal plane with leftward rotation of the probe (UE Ao Arch SAX view). Pulmonary venous return is not obstructed at any point. A catheter placed in the left internal jugular vein is seen in the vertical vein by 2D imaging. This video includes prominent electrocautery and Doppler mirror image artifacts. MPA main pulmonary artery (MP4 8294 kb)

In this ME 4-Ch view, a pulmonary venous confluence in an infant with total anomalous pulmonary return is shown adjacent to the left atrium (circular posterior structure) (MP4 722 kb)

Orthogonal view from that shown in Video 6.28. Color flow Doppler interrogation of the pulmonary venous confluence as displayed in the long axis. The blue flow away from the imaging transducer suggests an infradiaphragmatic course (MP4 425 kb)

ME 4-Ch view with clockwise transducer rotation showing anomalous direct drainage of a right lower pulmonary vein into the right atrium (MP4 359 kb)

ME 4-Ch view of a dilated coronary sinus (CS) in a patient with total anomalous pulmonary venous connection to the CS (MP4 738 kb)

Image in the mid esophagus at a transducer angle of 54° focusing on 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 (MP4 939 kb)

Corresponding image to that displayed in Video 6.32. Color flow Doppler across the right upper pulmonary vein reveals narrowing resulting from torsion of the pulmonary venous confluence to the left atrial anastomosis (MP4 191 kb)

ME 4-Ch view displaying the anastomotic connection between a pulmonary venous confluence and the left atrium in an infant with supracardiac total anomalous pulmonary venous connection. The anastomotic site appears widely patent by 2D imaging (MP4 713 kb)

Case #1. Sweep up to the upper esophagus to the level of the right pulmonary artery displaying the teardrop appearance of the superior vena cava (SVC) at the site of entry of an anomalous pulmonary vein. Note the presence of a central venous catheter in the SVC (MP4 3858 kb)

Case #1. Color flow Doppler of the image displayed in Video 6.35. The blue flow corresponds to the right pulmonary artery and the red flow to the anomalous right upper pulmonary vein as it enters the superior vena cava (MP4 3917 kb)

Case #1. Image obtained with clockwise probe rotation from the ME Asc Ao SAX view in the same patient depicted in Videos 6.35 and 6.36 showing the sinus venosus atrial septal defect and anomalous drainage of right upper pulmonary vein into the junction of the superior vena cava and right atrium (MP4 3060 kb)

Case #1. DTG Atr septal view with color flow Doppler confirms the presence of a sinus venosus atrial septal defect and associated anomalous drainage of a right upper pulmonary vein (MP4 609 kb)

Case #1. Initial postoperative TEE (repair using two-patch technique) in a modified ME view in the same patient depicted in Video 6.38 showing a patent pulmonary venous baffle with unobstructed flow by color flow mapping. Note atrial septal defect patch (MP4 2956 kb)

Case #1. Imaging of the superior vena cava in the same patient depicted in Video 6.39 in the DTG Atr Sept view with color flow Doppler. No color flow disturbance is observed; however, spectral interrogation was consistent with obstruction, which was confirmed by direct pressure measurement resulting in return to cardiopulmonary bypass (MP4 3472 kb)

Case #2. Initial preoperative TEE in the TG IVC/Hep veins view showing anomalous drainage of a right lower pulmonary vein (red flow) into a dilated inferior vena cava. The pulmonary vein appears smaller as compared to a dilated hepatic vein (MP4 1027 kb)

Case #2. Initial postoperative image in the patient depicted in Video 6.41 after bovine pericardial baffling of anomalous right lower pulmonary vein into the left atrium. The TG IVC/Hep veins view shows a mobile inferior vena cava portion of the baffle by 2D imaging (MP4 3779 kb)

Case #2. Corresponding TG IVC/Hep veins view to Video 6.42 shows unobstructed flow across the inferior vena cava portion of the baffle by color flow Doppler (red flow) (MP4 2201 kb)

Case #2. Preoperative TEE during second surgery with ME imaging at a transducer angle of 0° showing a pedunculated, mobile thrombus in the systemic venous aspect of the baffle (MP4 3699 kb)

Case #2. Imaging of the pulmonary venous aspect of the baffle in the patient displayed in Video 6.44 shows thickening and mild obstruction by color flow Doppler imaging (MP4 5879 kb)

Case #2. Postoperative TEE during second surgery for baffle revision with image obtained at a transducer angle of 74° shows no residual thrombus (MP4 1272 kb)

Case #2. Color flow Doppler corresponding to image displayed in Video 6.46 shows mild flow disturbance of the pulmonary venous end of the baffle (MP4 744 kb)

Case #2. Preoperative TEE during third surgical intervention displaying a sweep in the ME 4-Ch view and as the probe shaft is rotated rightward, showing the recurrence of a large, broad-based thrombus on the systemic and pulmonary venous aspects of the baffle in the same patient depicted in Videos 6.42–6.47 (MP4 12578 kb)

Case #2. Color flow imaging of the pulmonary venous pathway at a transducer angle of 91° obtained at the same time as the exam in this Video shows narrowing and aliasing consistent with recurrent obstruction (MP4 3926 kb)

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Tacy, T.A., Maskatia, S.A. (2021). Systemic and Pulmonary Venous Anomalies. In: Wong, P.C., Miller-Hance, W.C. (eds) Transesophageal Echocardiography for Pediatric and Congenital Heart Disease. Springer, Cham. https://doi.org/10.1007/978-3-030-57193-1_6

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