Assessing the Patient with Congenital Heart Disease

Abstract

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.

Electronic Supplementary Material Congenital heart disease (CHD) electronic supplementary material

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

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

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)

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)

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

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

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

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)

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)

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

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

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)

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)

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

Subcostal view of the atrioventricular septal (WMV 739 kb)

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

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

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

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)

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)

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)

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

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)

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)

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)

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)

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

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)

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)

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

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)

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)

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)

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)

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

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

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

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)

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