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Abnormalities of the Great Arteries

  • John SimpsonEmail author

Abstract

This chapter describes the sonographic features of major abnormalities of the outflow tracts including transposition of the great arteries, common arterial trunk, tetralogy of Fallot and double outlet right ventricle as well as aortic and pulmonary valve stenosis. The sonographic features of each lesion are illustrated and contrasted with the normal anatomy of the fetal cardiac outflow tracts. The associations of the different abnormalities and the approach to postnatal management are also discussed.

Keywords

Fetal heart Outflow tracts Great arteries Congenital heart disease 

Supplementary material

Video 8.1

Normal four chamber view in fetus with transposition of the great arteries (AVI 1777 kb) (AVI 54759 kb)

Video 8.2

Sonographic sweep showing the branching pulmonary artery arising from the left ventricle. On sweeping more cranially, the aorta can be seen to arise from the right ventricle. The normal 3VT appearance is not evident (AVI 45524 kb)

Video 8.3

In this video, the branching PA is seen more posteriorly followed by visualisation of the aorta more anteriorly as the ultrasound probe is angulated superiorly (AVI 66368 kb)

Video 8.4

Sonographic sweep utilising colour Flow Doppler to illustrate the pulmonary artery arising from the LV and, more cranially, the aorta arising from the RV (AVI 43956 kb)

Video 8.5

Parallel arrangement of the great arteries with the aorta anterior is evident in this video. There is loss of the normal “crossing” pattern of the great arteries (AVI 52459 kb)

Video 8.6

This is a sagittal projection of transposition of the great arteries. The arteries arise in parallel from the heart with the aorta anterior. Note that the aortic arch is superior to the ductal arch and that the plan of the two arches is similar so that they my both be seen in the same sonographic cut, in contrast to the normal heart (MP4 3680 kb)

Video 8.7

A large perimembranous VSD is clearly evident on the four chamber view in this fetus with TGA (AVI 18363 kb)

Video 8.8

In this video the left panel show that the great arteries arise in parallel but that the pulmonary artery is significantly smaller than the aorta. The right panel demonstrates aliasing of colour flow in the pulmonary artery due to pulmonary valve stenosis. There is also a red jet of retrograde flow in the arterial duct (AVI 24423 kb)

Video 8.9

Common arterial trunk: This video shows the sweep from four chamber view superiorly towards the common arterial trunk which overrides a ventricular septal defect (MP4 2325 kb)

Video 8.10

Short axis view of a common arterial trunk showing the origin of the branch pulmonary arteries as well as continuation of the trunk into a right sided aortic arch (AVI 1777 kb)

Video 8.11

The common arterial trunk is shown in short axis along with the origin of the branch pulmonary arteries (MP4 2361 kb)

Video 8.12

Dual display of greyscale and colour flow Doppler image of the common arterial trunk arising predominantly from the RV. The left pulmonary artery can be seen arising from the common arterial trunk (MP4 2450 kb)

Video 8.13

Truncal valve stenosis and regurgitation. There is marked aliasing of blood flow across the truncal valve, indicating stenosis of the truncal valve. There is also truncal valve regurgitation as evidenced by the red jet of blood flowing back from the trunk into the RV (MP4 2065 kb)

Video 8.14

Rotation of the cardiac apex to the left is illustrated in a fetus with tetralogy of Fallot (MP4 2377 kb)

Video 8.15

Sweep from the four chamber view to the aorta demonstrating overriding of the aorta. There is loss of the normal continuity between the anterior wall of the aorta and the ventricular septum (AVI 31527 kb)

Video 8.16

Short axis views illustrating relative hypoplasia of the pulmonary arteries compared to the aorta. The pulmonary arteries are confluent (AVI 31039 kb)

Video 8.17

This sweep shows all of the key features of tetralogy of Fallot including a normal four chamber view, overriding of the aorta and normal antero-posterior orientation of the main pulmonary artery. The aortic arch is left sided in this example (MP4 8918 kb)

Video 8.18

This sonographic view of the upper mediastinum shows a right sided aortic arch and retrograde flow into the pulmonary arteries through a right sided arterial duct (WMV 3747 kb)

Video 8.19

This is an oblique view illustrating a short axis view of the aortic valve. The VSD is seen showing continuity between the tricuspid valve and the aorta (perimembranous VSD) and anterior deviation of the outlet septum into the right ventricular outflow tract (AVI 29431 kb)

Video 8.20

This dual projection image shows overriding of the aorta above a ventricular septal defect which is confirmed on colour flow Doppler (AVI 31283 kb)

Video 8.21

This sweep from the four-chamber view shows normal four-chamber appearances, overriding of the aorta, no demonstrable pulmonary arteries and a left sided aortic arch (AVI 14842 kb)

Video 8.22

The aorta arises astride a ventricular septal defect and the pulmonary arteries are confluent but severely hypoplastic (AVI 54380 kb)

Video 8.23

Use of colour flow Doppler in this fetus demonstrates small but confluent branch pulmonary arteries. There is retrograde (red) colour flow in the right pulmonary artery (AVI 60868 kb)

Video 8.24

Three major aortopulmonary arteries can be seen arising from the descending aorta in this view. The precise course of such arteries within the lungs is extremely difficult to determine during fetal life (MP4 2840 kb)

Video 8.25

The cardiac axis is rotated to the left in this fetus with tetralogy of Fallot with absent pulmonary valve (AVI 14884 kb)

Video 8.26

This sweep from the four chamber view illustrates the rudimentary nature of the pulmonary valve and the marked dilation of the branch pulmonary arteries. Note that the descending aorta is to the right of the spine (right aortic arch) (AVI 61148 kb)

Video 8.27

Sonographic sweep from four-chamber view showing aortic override, abnormal pulmonary valve and dilated branch pulmonary arteries (MP4 8720 kb)

Video 8.28

Early gestation fetus (17 weeks) showing dramatically enlarged and pulsatile branch pulmonary arteries (AVI 27271 kb)

Video 8.29

Use of colour flow Doppler clearly illustrates the to and fro nature of blood flow across the rudimentary pulmonary valve (MP4 1365 kb)

Video 8.30

The antegrade flow across the pulmonary valve ring is blue and aliased, contrasting with the severe regurgitation illustrated by the retrograde flow from the pulmonary arteries into the right ventricle (red colour) (AVI 52408 kb)

Video 8.31

Double outlet right ventricle illustrated by sweep from the four-chamber view to the great arteries. The aorta is seen first, followed by the pulmonary artery. Both great arteries arise entirely from the right ventricle (MP4 7131 kb)

Video 8.32

In this example, the fetus is spine up with a relatively parallel arrangement of the great arteries with the aorta anterior (transposed-type position). The pulmonary vale is in closest relationship to the ventricular septal defect (WMV 941 kb)

Video 8.33

This fetus has a complete atrioventricular septal defect with double outlet right ventricle. In this example the aorta is anterior to the pulmonary artery which is also significantly smaller than the aorta (AVI 1673 kb)

Video 8.34

Spatio-temporal image correlation (STIC) in a fetus with double outlet right ventricle. This multi-slice view shows sequential sonographic cuts from inferiorly (lower panels) to superiorly (upper panels) in the fetal heart to assist in understanding the spatial orientation in this lesion (AVI 442 kb)

Video 8.35

STIC representation of the ventricles and great arteries in fetal double outlet right ventricle. The depth of field enhances understanding of the relative position of the vessels (AVI 138 kb)

Video 8.36

Aortic valve stenosis. In this example, left ventricular function is good but the aortic valve appears dysplastic and restricted in its motion (AVI 14175 kb)

Video 8.37

In this oblique sagittal view of a fetus with mild aortic valve stenosis, there is poststenotic dilation of the ascending aorta (AVI 14226 kb)

Video 8.38

Short axis view of the aortic valve illustrating that this is bicuspid. The morphology of the valve may only be visualised with good image quality, typically in the late second trimester and beyond (AVI 25362 kb)

Video 8.39

Classical view of critical aortic valve stenosis. The left ventricle is dilated, echogenic and poorly contractile. The excursion of the mitral valve is reduced. The echogenicity of the left ventricle (endocardial fibroelastosis) is most marked in the ventricular septum (AVI 30050 kb)

Video 8.40

Four-chamber view of critical aortic valve stenosis with colour flow Doppler. The left ventricle is dilated and contracts poorly. There is significant mitral valve regurgitation and tricuspid valve regurgitation (AVI 14613 kb)

Video 8.41

Four-chamber view of critical aortic valve stenosis showing left to right flow of blood at atrial level, the reverse of the normal pattern (MP4 2352 kb)

Video 8.42

This sagittal view of the aortic arch shows retrograde flow in the aortic arch, confirming critical left heart obstruction (MP4 1486 kb)

Video 8.43

Critical aortic stenosis at 20 weeks gestational age with a dilated poorly functioning left ventricle (AVI 2617 kb)

Video 8.44

This is the same fetus as Video 8.43 but now at 29 weeks gestational age. The right ventricle now forms the cardiac apex and the size of the left ventricle is now smaller than the right ventricle (AVI 2686 kb)

Video 8.45

Four chamber view of the same fetus as videos 8.43 and 8.44, now at 34 weeks gestational age. The left ventricle is now severely hypoplastic, echogenic and does not contract. These findings are almost identical to classical hypoplastic left heart syndrome (AVI 903 kb)

Video 8.46

Pulmonary atresia with intact ventricular septum. The right ventricle in this example is dilated with poor function. There is little excursion of the tricuspid valve. In contrast left ventricular function is normal and the mitral valve opens well (MP4 4049 kb)

Video 8.47

The right ventricle is hypertrophied with reduced cavity size. There is significant tricuspid valve regurgitation which had a Doppler velocity in excess of 4 m/s confirming elevated right heart pressures. There is also mild mitral valve regurgitation (AVI 27994 kb)

Video 8.48

This oblique view shows normal opening of the aortic valve. In contrast, the pulmonary valve has restricted motion during systole (AVI 26100 kb)

Video 8.49

Critical pulmonary valve stenosis. The is turbulent flow across the pulmonary valve shown and colour flow aliasing despite a high Doppler scale. The Doppler velocity was in excess of 3 m/s (AVI 28612 kb)

Video 8.50

Pulmonary atresia with intact ventricular septum. This four-chamber view shows severe hypoplasia and hypertrophy of the right ventricle with an RV cavity which is almost obliterated. Colour flow Doppler confirms normal filling of the left ventricle but virtually no flow into the right ventricle. Towards the apex of the right ventricle there are colour foow jets over the myocardium consistent with RV to coronary communications (MP4 3470 kb)

Video 8.51

This video clip shows normal (blue) flow out of the left ventricle. There is retrograde filling of the branch pulmonary arteries (MP4 3885 kb)

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Foundation TrustLondonUK

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