Assessing the Patient with Congenital Heart Disease

  • John M. SimpsonEmail author
  • Karolina Hall


The major focus of this chapter is on echocardiography as this is the dominant modality used during clinical review but the importance of other modalities such as magnetic resonance imaging (MRI) or Computed Tomography (CT) will be discussed where relevant. Multimodality imaging has become the norm in the assessment of the patient with CHD particularly for surgical planning, follow-up after surgery and in older patients with suboptimal acoustic windows.


Echocardiography Congenital heart disease Three-dimensional echocardiography Imaging Paediatrics Transposition of the great arteries Fontan Tetralogy of Fallot 

Supplementary material

Video 32.1

a The left atrial appendage has a finger like shape (AVI 6981 kb)

Video 32.1

b The right atrial appendage has a typical triangular shape and a broad base (AVI 10834 kb)

Video 32.2a

Usual arrangement of the abdominal vessels. The liver is seen to the right. The aorta descends to the left of the spine and the inferior vena cava is more anterior and rightwards (AVI 3362 kb)

Video 32.2b

Mirror image atrial arrangement. The liver is on the left. The aorta descends to the right of the spine and the inferior vena cava is anterior and to the left (AVI 6772 kb)

Video 32.2c

Left isomeric abdominal situs view. Note the absent IVC and dilated hemi-azygous left and below the abdominal Aorta (MP4 666 kb)

Video 32.2d

Right isomeric abdominal situs view. Note the midline liver (AVI 5877 kb)

Video 32.3a

This video shows the normal drainage of the inferior vena cava to the right atrium (MP4 1168 kb)

Video 32.3b

In this example the inferior vena cava is interrupted and the hemi-azygous vein continues posterior to the descending aorta. The descending aorta is the pulsatile red flow and the azygous flow is continuous and blue (MP4 6267 kb)

Video 32.3c

Sagittal cut of the abdominal aorta along the left of spine. This view demonstrates the pulsatile nature of a normal Aorta upstream from this point (AVI 19095 kb)

Video 32.4a

This video illustrates blood returning to the left atrium through the pulmonary veins (AVI 4201 kb)

Video 32.4b

This video shows right to left shunting of blood at atrial level and a confluence behind the left atrium (AVI 3811 kb)

Video 32.5

The video shows the four chamber view of the normal heart illustrating the normal differential insertion of the atrioventricular valves and the attachments of these valves (AVI 20213 kb)

Video 32.6

AV discordance in the same heart shown in Fig. 32.7. There is complete atrioventricular block which is common in this pathology (AVI 7435 kb)

Video 32.7a

Apical four chamber view of a complete AVSD (MP4 445 kb)

Video 32.7b

Subcostal view of the atrioventricular septal (WMV 739 kb)

Video 32.7c

Three-dimensional echocardiogram of an atrioventricular septal defect en face to the ventricular aspect of the valve (MP4 147 kb)

Video 32.7d

Three-dimensional transoesophageal echocardiogram of an atrioventricular septal defect en face from the ventricular apex (WMV 380 kb)

Video 32.8

Transthoracic three-dimensional echocardiogram of a double orifice mitral valve obtained from a parasternal short axis view (WMV 1114 kb)

Video 32.9a

Apical four chamber view of Ebstein’s malformation of the TV. The septal leaflet of the TV is displaced apically, so the normal pattern of differential insertion of the mitral and tricuspid valves is completely lost. The “atrialised” portion of the right ventricle is shown (AVI 12874 kb)

Video 32.9b

Three-dimensional projection of the apical four chamber view of Ebstein’s anomaly. Note the apical displacement of the inferior TV leaflet and the long sail-like anterosuperior leaflet (AVI 2769 kb)

Video 32.9c

Subcostal oblique three-dimensional echocardiographic view of the TV in Ebstein’s anomaly. The TV is displaced towards the RV apex ( ) but is also rotated superiorly towards the RVOT (arrows) (AVI 3666 kb)

Video 32.10

In the normal heart the RVOT wraps around the aorta which is seen in short axis (AVI 48232 kb)

Video 32.11

Parasternal long axis view of simple transposition of the great arteries. The great arteries run parallel to each other with the aorta anterior to the pulmonary artery (MP4 291 kb)

Video 32.12

A high sagittal view under the left mid-clavicle to demonstrate the proximal main pulmonary artery and the distal aortic arch, joined by a patent arterial duct, visible here as a red flare on colour Doppler (AVI 11968 kb)

Video 32.13a

In a left sided aortic arch the first branch is the innominate artery which heads to the right side and bifurcates into the right carotid and right subclavian arteries (AVI 5073 kb)

Video 32.13b

In a right sided aortic arch, there is usually mirror image branching so that the first vessel heads to the left as is a left sided innominate artery which bifurcates into the left carotid and left subclavian arteries (WMV 426 kb)

Video 32.14a

This view permits visualisation of all four pulmonary veins to the left atrium. (AVI 4661 kb)

Video 32.14b

A sweep in the suprasternal coronal ‘crab’ view of the pulmonary veins but with supracardiac total anomalous pulmonary venous drainage via an ascending vein (red on colour Doppler) that drains into the innominate vein and eventually the SVC (AVI 45620 kb)

Video 32.15a

Subcostal oblique view of tetralogy of Fallot. The aorta is much larger than the pulmonary artery and there is muscular subpulmonary obstruction due to antero-superior deviation of the outlet septum (AVI 5195 kb)

Video 32.15b

Parasternal long axis view demonstrating a ventricular septal defect (*) and aortic override typical of Tetralogy of Fallot (MP4 705 kb)

Video 32.15c

Parasternal short-axis view demonstrating the large perimembranous ventricular septal defect (*), the anterosuperior deviation of the infundibular septum (>), the consequent infundibular stenosis and the hypoplastic pulmonary valve typical of Tetralogy of Fallot (MP4 536 kb)

Video 32.16a

High parasternal short-axis showing the branch pulmonary arteries positioned anteriorly to the aorta following the Lecompte Manoeuvre during the arterial switch operation. In larger patients it becomes difficult to see the branch pulmonary arteries which are better appreciated with the use of colour flow Doppler (AVI 6388 kb)

Video 32.16b

Parasternal long axis view of adolescent patient following an arterial switch operation. There is mild central aortic regurgitation. The aortic annulus is dilated as is the aortic root (38 mm, marked by calipers) with loss of the normal anatomy of the sinotubular junction (AVI 16300 kb)

Video 32.17a

Apical four chamber view showing the pulmonary veins being diverted through the pulmonary venous baffle towards the tricuspid valve and systemic right ventricle (AVI 9987 kb)

Video 32.17b

Short axis view showing the large right ventricle and flattened ventricular septum following the Senning operation (AVI 23793 kb)

Video 32.17c

Three-dimensional transoesophageal echocardiogram showing the systemic venous baffles viewed from the left side (AVI 14498 kb)

Video 32.18

■■■ (WMV 373 kb)

Video 32.19a

The continuous flow from the SVC into the branch pulmonary arteries is well seen (AVI 8284 kb)

Video 32.19b

This subcostal view shows flow from the inferior vena cava into the lateral tunnel which permits blood to flow from the inferior vena cava to the branch pulmonary arteries (AVI 4782 kb)

Video 32.19c

Gadolinium enhanced MRI of the total cavopulmonary connection showing the IVC, SVC, lateral tunnel and branch pulmonary arteries (WMV 2677 kb)


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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Congenital Heart DiseaseEvelina London Children’s HospitalLondonUK

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