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Evaluation of Conotruncal Abnormalities

  • Laura M. Mercer-Rosa
  • Meryl S. CohenEmail author
Chapter

Abstract

Conotruncal malformations encompass a group of congenital heart defects with abnormal ventriculo-arterial connections. The conotruncal abnormalities are often seen in association with other cardiac defects, and the intracardiac anatomy can be quite complex. Cardiac surgery is almost always required to repair the anatomic abnormalities and restore normal physiology. As such, transesophageal echocardiography (TEE) is often required in the intraoperative setting in order to evaluate and confirm preoperatively the cardiac abnormalities, and to assess the cardiac repair postoperatively. Moreover, patients with a conotruncal malformation (operated and sometimes unoperated) will survive into adulthood, and in the ambulatory setting, TEE can provide important anatomic and physiologic information superior to that available by transthoracic echocardiography. This chapter discusses the TEE evaluation of the most common and important conotruncal defects, including tetralogy of Fallot, double outlet right ventricle, truncus arteriosus, transposition of the great arteries (also known as complete, or D-transposition of the great arteries), and congenitally corrected transposition of the great arteries.

Keywords

Transesophageal echocardiography Heart defects Congenital Tetralogy of Fallot Double outlet right ventricle Transposition of the great arteries, complete Truncus arteriosus Transposition of the great arteries, corrected 

Supplementary material

Video 12.1

Tetralogy of Fallot: Mid esophageal four chamber view showing right ventricular hypertrophy, the large malalignment ventricular septal defect, and overriding aorta. LA left atrium, LV left ventricle, RV right ventricle (MPG 2454 kb)

Video 12.2

Tetralogy of Fallot: Modified mid esophageal right ventricular inflow-outflow view (multiplane angle about 90°) showing the malalignment ventricular septal defect (VSD), as well as the narrowing of right ventricular outflow due to a malaligned conal septum. Ao aorta, MPA main pulmonary artery, PV pulmonary valve, RV right ventricle (MPG 3080 kb)

Video 12.3

Tetralogy of Fallot: Deep transgastric long axis and sagittal views that simulate transthoracic subcostal coronal and sagittal views, showing the anteriorly malaligned infundibular septum producing subpulmonary stenosis. The spectral Doppler tracing shows the typical “dagger” shape seen with subpulmonary stenosis. Color Doppler displays the aliased flow across the right ventricular outflow tract and right to left shunting across the ventricular septal defect. Ao aorta, LV left ventricle, PA pulmonary artery, RA right atrium, RV right ventricle (MPG 13660 kb)

Video 12.4

Mid esophageal four chamber view demonstrating right ventricular hypertrophy in a patient with tetralogy of Fallot. Also note the presence of a ventricular septal defect patch, in good position (MOV 10811 kb)

Video 12.5

Preoperative study in a patient with tetralogy of Fallot. A prominent anterior descending coronary artery (arrow) arises from the right coronary artery (RCA) and courses anterior to the right ventricular outflow tract, thereby precluding a transannular patch. Ao aorta, PA main pulmonary artery (MPG 6644 kb)

125052_1_En_12_MOESM6_ESM.mov (995 kb)
Video 12.6 Modified mid esophageal long axis view, with multiplane angle 87° in a patient with tetralogy of Fallot demonstrating a residual ventricular septal defect. The defect is located in the superior aspect of the patch just under the aortic valve. Shunting is seen into the right ventricular outflow tract (MOV 994 kb)
Video 12.7

Upper esophageal aortic arch short axis with counterclockwise rotation, demonstrating a significant left pulmonary artery stenosis in this patient with tetralogy of Fallot who underwent a complete repair, including repair of discontinuous pulmonary arteries. Spectral continuous wave Doppler tracing shows the significant gradient across the stenotic area. Desc Ao descending aorta, LPA left pulmonary artery, MPA main pulmonary artery (MPG 9138 kb)

Video 12.8

Residual right ventricular outflow tract narrowing is seen in a patient after tetralogy of Fallot repair in the mid esophageal right ventricular inflow-outflow view, with slight probe withdrawal to display the outflow tract more clearly (MOV 8121 kb)

Video 12.9

Mid esophageal aortic valve long axis view demonstrating an unobstructed left ventricular outflow tract after repair of tetralogy of Fallot (MOV 5809 kb)

Video 12.10

Mid esophageal four chamber view with probe withdrawn to evaluate the left ventricular outflow tract demonstrates that there is no residual VSD seen by color Doppler interrogation after tetralogy of Fallot repair. In addition, flow is laminar into the left ventricular outflow tract (MOV 6160 kb)

Video 12.11

Mid esophageal four chamber view in a patient who has undergone tetralogy of Fallot and atrioventricular canal repair. A large ventricular septal defect patch is seen. Minimal residual atrioventricular valve regurgitation is seen across the right and left atrioventricular valves (MOV 6885 kb)

Video 12.12

Severe tricuspid regurgitation prompted tricuspid valve replacement in this adult long after tetralogy of Fallot repair. This is a short Video 12.sweep that starts in a mid esophageal bicaval view, and as the probe is rotated counterclockwise (leftwards) to a modified mid esophageal right ventricular inflow-outflow view (multiplane angle 97°), the prosthetic valve appears in cross section (MOV 9588 kb)

Video 12.13

Mid esophageal four chamber view in a patient with double outlet right ventricle, posterior malalignment of the conal septum, and subpulmonary ventricular septal defect, with the probe withdrawn towards the base of the heart. The aorta and pulmonary artery (PA) come into view, both arising from the right ventricle (RV). The PA is smaller than the aorta because of the conal septal malalignment and pulmonary outflow tract stenosis (MOV 11699 kb)

Video 12.14

Mid esophageal aortic valve long axis view in the patient in Video 12.13 with double outlet right ventricle, posterior malalignment of the conal septum with subpulmonary ventricular septal defect highlighting the posterior deviation of the conal septum resulting in severe sub pulmonary stenosis. The conal septum is also hypertrophied (MOV 11746 kb)

Video 12.15

Double outlet right ventricle with pulmonary outflow tract stenosis. Mid esophageal four chamber view shows a large ventricular septal defect (arrow). With probe withdrawal and anteflexion, the semilunar valves are visualized in a side-by-side orientation. However the pathway from left ventricle (LV) to aortic valve (AoV) is not clearly shown. Rotation of multiplane angle to about 90° and sweep from right to left shows right atrium (RA) and right ventricle (RV) as well as AoV, but it is still unclear whether the pathway from LV to AoV is unobstructed. The deep transgastric long axis view shows that this pathway is unobstructed; it also shows the origin of both great arteries from the RV. This patient successfully underwent a patch closure of the ventricular septal defect and relief of pulmonary outflow tract stenosis. LA left atrium, PV pulmonary valve (MPG 12812 kb)

Video 12.16

Left juxtaposition of the atrial appendages in a patient with double outlet right ventricle and pulmonary stenosis. This Video was obtained with a mid esophageal aortic valve short axis view. Note how the right atrial appendage (RAA) crosses posterior to the great arteries to lie just anterior to the left atrial appendage (LAA). AoV aortic valve, RA right atrium, PV pulmonary valve (MPG 4432 kb)

Video 12.17

Mid esophageal sagittal (90°) clockwise sweep (from left to right) in the patient from Videos 12.13 and 12.14 with double outlet right ventricle, posterior malalignment of the conal septum and subpulmonary ventricular septal defect after the Nikaidoh procedure (aortic translocation). The translocation of the aorta has placed the aortic valve closer to the left ventricle, improving left ventricle to aortic valve alignment and reducing the possibility of subaortic obstruction once the ventricular septal defect has been closed by a patch. This video demonstrates the “physiologic” repair achieved by baffling the ventricular septal defect to the aorta in its new position, and the right ventricle to pulmonary artery conduit (MOV 9880 kb)

Video 12.18

Truncal valve seen en face from a modified mid esophageal aortic valve short axis view (angle 0°). This shows a quadricuspid truncal valve with thickened edges and a central area of noncoaptation associated with a small amount of valvar regurgitation (MPG 2940 kb)

Video 12.19

Truncus arteriosus type “1½ ”. From the mid esophageal ascending aortic short axis, the right pulmonary artery (RPA) and left pulmonary artery (LPA) origins are seen immediately adjacent to each other, arising from the posterior aspect of the trunk (TRUN). Color flow demonstrates unobstructed flow across the origin of the branch pulmonary arteries. From the mid esophageal aortic valve long axis view the posterior origin of the pulmonary arteries (arrow) is also seen and the truncal valve shown to override the ventricular septal defect. Trace truncal valve regurgitation is demonstrated. Ao ascending aorta, LA left atrium, LV left ventricle, RV right ventricle (MPG 12542 kb)

Video 12.20

Postop truncus repair, seen from mid esophageal four chamber view with probe anteflexion. The ventricular septal defect patch is shown by the arrow; no residual defect is present. A small amount of truncal valve regurgitation is seen. LV left ventricle, RV right ventricle (MPG 3370 kb)

Video 12.21

Postop truncus arteriosus repair, seen from the mid esophageal aortic valve long axis view, multiplane angle about 75°, with imaging and color flow Doppler. The ventricular septal defect patch is seen as well as the takeoff of the conduit (COND) from the right ventricle (RV) to the pulmonary artery. LV left ventricle (MPG 4978 kb)

Video 12.22

Transposition of the great arteries. Using a mid esophageal aortic valve short axis view, both semilunar valves are seen en face, with the aortic valve (AoV) anterior and rightward to the pulmonary valve (PV). LA left atrium, RA right atrium (MPG 2896 kb)

Video 12.23

Origins of the right and left coronary arteries from the opposite sinuses in transposition of the great arteries, as seen from the mid esophageal aortic valve short axis view. This coronary pattern is the most common type seen in this cardiac defect. Ao aorta, PA pulmonary artery (MPG 5960 kb)

Video 12.24

Transposition of the great arteries, as viewed from the mid esophageal four chamber view. With anteflexion and slight probe withdrawal, both great arteries are seen in parallel, with the aorta (Ao) arising from the right ventricle (RV), and the pulmonary artery (PA) from the left ventricle. Color flow Doppler shows continuous flow in the PA from ductal shunting (MPG 4938 kb)

Video 12.25

Transposition of the great arteries, as viewed from a mid esophageal right ventricular inflow-outflow/long axis view, multiplane angle about 90°. Both great arteries are seen in parallel, with the aorta arising from the right ventricle, and the pulmonary artery from the left ventricle. Ao aorta, LA left atrium, LV left ventricle, PA pulmonary artery, RA right atrium, RV right ventricle. Color flow Doppler shows continuous flow in the PA (MPG 7050 kb)

Video 12.26

Transposition of the great arteries, with a large ventricular septal defect and pulmonary outflow obstruction, as seen from modified mid esophageal four chamber, bicaval, and long axis views. A right to left sweep is performed from the bicaval to long axis view, showing the atrioventricular valves, ventricles, and ventricular septal defect. Posteriorly malaligned infundibular septum produces prominent subpulmonary narrowing, and turbulence is seen by color flow Doppler across the pulmonary outflow tract. Ao aorta, LA left atrium, LV left ventricle, PA pulmonary artery, PV pulmonary valve, RA right atrium, RV right ventricle, SVC superior vena cava (MPG 14052 kb)

Video 12.27

Postoperative study following arterial switch operation. From the mid esophageal long axis view, the neo-aortic valve is seen with a small amount of regurgitation. The aortic anastomosis is shown by the arrow. Withdrawal of the probe to the mid esophageal ascending aorta long axis view shows the pulmonary artery anastomosis (arrow); the branch pulmonary arteries arise just above (superior to) the anastomosis. In this video, the right pulmonary artery is seen. Ao aorta, LA left atrium, LV left ventricle, MPA main pulmonary artery, RPA right pulmonary artery, RV right ventricle (MPG 12560 kb)

Video 12.28

Postoperative study following arterial switch operation, showing the branch pulmonary arteries from the upper esophageal pulmonary artery long axis view. A Lecompte maneuver was performed so that the main and branch pulmonary arteries are situated anterior to the aorta (Ao). Color flow Doppler demonstrates flow in both the main and branch pulmonary arteries. LPA left pulmonary artery, RPA right pulmonary artery (MPG 4122 kb)

Video 12.29

Mid esophageal right ventricular inflow-outflow view with the probe advanced toward the liver in an adult who has undergone an atrial switch operation for transposition of the great arteries. The color interrogation demonstrates flow from the pulmonary venous channel to the systemic venous channel (left to right shunt) (MOV 1276 kb)

Video 12.30

Mid esophageal right ventricular inflow-outflow view in a patient with an atrial switch and a baffle leak. This video is obtained during device deployment. The image demonstrates an Amplatzer device just prior to release (MOV 3425 kb)

Video 12.31

Mid esophageal four chamber view of a patient after atrial switch operation demonstrating the pulmonary venous channel as it makes its way to the tricuspid valve. In this patient, the pathway is unobstructed (MOV 3575 kb)

Video 12.32

Mid esophageal bicaval view with probe turned clockwise toward the patient’s right side. This image is from a patient who has superior systemic venous limb obstruction after an atrial switch operation. In this view, both inferior and superior limbs of the systemic venous channel are seen with the pulmonary venous channel coursing in between. Pacing wires are seen in the superior limb. If the probe is turned further clockwise, the right pulmonary veins will be profiled (MOV 4143 kb)

Video 12.33

Modified mid esophageal right ventricular inflow-outflow view using color Doppler in the same patient as Video 12.32 after atrial switch operation, with superior limb obstruction. Aliasing of the color flow is seen in the superior limb of the channel (MOV 1309 kb)

Video 12.34

Congenitally corrected transposition of the great arteries. A mid esophageal four chamber view shows inferior displacement of the septal leaflet of the left sided tricuspid valve, compared with the right sided mitral valve. Incomplete coaptation of this valve results in a mild to moderate degree of tricuspid insufficiency. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle (MPG 6474 kb)

Video 12.35

Congenitally corrected transposition of the great arteries. Using the mid esophageal long axis view, the left sided tricuspid valve is seen, with significant regurgitation. Ao aorta, LA left atrium, PA pulmonary artery, RV right ventricle (MPG 5816 kb)

Video 12.36

Mid esophageal four chamber view shows a large perimembranous ventricular septal defect (arrow) in a patient with congenitally corrected transposition of the great arteries. Note the left sided tricuspid valve chordal attachment to ventricular septum, as well as the presence of a moderator band in the left sided right ventricle (RV). LA left atrium, LV left ventricle, RA right atrium (MPG 3428 kb)

Video 12.37

Congenitally corrected transposition of the great arteries. Transgastric mid short axis view shows the inverted ventricles in cross section. Note the smooth walled septal surface in the left ventricle (LV), and the prominent moderator band in the right ventricle (RV) (MPG 3158 kb)

Video 12.38

Congenitally corrected transposition of the great arteries, as seen from the mid esophageal window in an approximately 90° sagittal plane. There is a “windsock” aneurysm of tissue (arrow) originating from the anterior leaflet of mitral valve and protruding into the pulmonary outflow tract, just below the pulmonary valve. In addition fibrous tissue from the mitral valve extends across the outflow tract and attaches to the ventricular septum. Neither of these was obstructive. A pulmonary artery band is seen in the main pulmonary artery, with flow acceleration noted across it. LA left atrium, LV left ventricle, PA pulmonary artery, RA right atrium (MPG 5940 kb)

Video 12.39

Deep transgastric long axis view in a patient with congenitally corrected transposition of the great arteries and ventricular septal defect, in whom a pulmonary artery band (Band) was placed. The transducer first visualizes the right ventricle (RV) and ascending aorta (Ao). Further probe anteflexion and advancement is required to visualize the most posterior pulmonary artery (PA). Significant aliasing is noted across the band, and spectral Doppler calculated gradient is 84 mmHg. Note the excellent angle for Doppler interrogation across the band. LV left ventricle, RA right atrium, RV right ventricle (MPG 9990 kb)

Video 12.40

Mid esophageal four chamber view in a patient with congenitally corrected transposition of the great arteries and ventricular septal defect. A sweep is performed in which the probe is simultaneously anteflexed and withdrawn to a view approximating a mid esophageal aortic valve short axis view. During this sweep, the aortic valve is seen to arise from the left sided right ventricle (RV), and there is subaortic conal muscle seen, along with discontinuity between the left sided tricuspid valve and aortic valve (AoV). At the same time, the pulmonary valve (PV), which arises from the right sided left ventricle (LV), is shown to have fibrous continuity with the right sided mitral valve. The coronary arteries are clearly seen arising from the anterior aorta. LA left atrium, RA right atrium (MPG 9066 kb)

Video 12.41

Mid esophageal long axis view in congenitally corrected transposition of the great arteries, showing the parallel course of both aorta (Ao) and pulmonary artery (PA) as they arise from the heart. In this view, the ventriculoarterial discordance, and parallel arrangement of the great arteries, is very similar to that seen in D-transposition of the great arteries. Thus complementary views and sweeps are necessary to determine visceroatrial situs and atrioventricular connections. LA left atrium (MPG 4334 kb)

Video 12.42

Deep transgastric long axis view in congenitally corrected transposition of the great arteries. With the probe advanced in the stomach to point the tip more posteriorly, the pulmonary artery (PA) is seen arising from the left ventricle (LV). The right atrium (RA) is also seen emptying into the LV. There is unobstructed flow across the pulmonary outflow tract. The probe is then withdrawn slightly, pointing the tip more anteriorly, to visualize the anterior aorta (Ao) arising from the right ventricle (RV). Flow across the aorta is also unobstructed. Note the moderator band present in the RV (MPG 10416 kb)

Video 12.43

Mid esophageal sagittal sweep in congenitally corrected transposition of the great arteries. Starting with the mid esophageal bicaval view, the left atrium (LA), right atrium (RA), mitral valve and left ventricle (LV) are noted. The transducer is rotated counterclockwise (leftward) to visualize further the LV and pulmonary artery (PA). With further leftward probe rotation, the LA, tricuspid valve, right ventricle (RV) and aorta (Ao) are visualized. RAA right atrial appendage, SVC superior vena cava (MPG 9256 kb)

Video 12.44

Same patient as Video 12.38. The pulmonary artery band is clearly seen well above the pulmonary valve, and color flow Doppler demonstrates significant aliasing across the band. LV left ventricle, PA pulmonary artery, RA right atrium, RV right ventricle (MPG 3578 kb)

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© Springer-Verlag London 2014

Authors and Affiliations

  1. 1.Division of Cardiology, Department of PediatricsThe Children’s Hospital of Philadelphia, Perelman School of Medicine at the University of PennsylvaniaPhiladelphiaUSA

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