Abstract
Congenital malformations of the heart affect at least 1% of newborn infants. Without intervention, the prognosis for more complex forms is poor. Over the last few decades, advances in paedriatic cardiology and cardiac surgery have significantly improved patient management, and the majority of patients now survive into adulthood.1, 2 This has led to new challenges as increasing numbers of congenital heart patients pass into the care of adult cardiac services. The need for expert knowledge to appropriately investigate the variable cardiovascular anatomy and pathophysiology and to manage this patient group has led to the expanding cardiological subspecialty of adult congenital heart disease (ACHD). This also poses new challenges with regard to cardiac imaging.
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Video 30.1
(a) Secundum ASD. Apical four-chamber view of a secundum ASD. Notice the central position of the ASD within the intra-atrial septum and the T-signs at the margins. The right ventricle is dilated. (b) Secundum ASD. Apical four-chamber view of a secundum ASD with colour-flow imaging. Notice the large left-to-right shunt across the intra-atrial septum.
Video 30.2
Primum ASD. Apical four-chamber view of a primum atrial septal defect. The defect is located in the inferior part of the intra-atrial septum. Note that both AV-valves are implanted at the same level. There was an abnormal left AV-valve.
Video 30.3
(a) Perimembranous VSD. Parasternal short-axis view with on 2-D imaging a large perimembranous VSD. (b) Colour-Doppler image of a perimembranous VSD.
Video 30.4
Atrio-ventricular septum defect. Apical four-chamber view. Notice the combination of a primum atrial septal defect with an inlet VSD and a single AV-valve between the atria and the ventricles.
Video 30.5
Coarctation of the aorta. Notice the presence of a localized coarctation in this patient with continuous flow pattern in the juxtaductal region.
Video 30.6
Double-chambered right ventricle. Sub-costal view where the hypertrophied muscle bundle dividing the right ventricle into a high pressure and low pressure part with the presence of right ventricular outflow tract obstruction as can be seen on the colour image.
Video 30.7
Ebstein’s anomaly. Apical four-chamber view of patient with Ebstain’s anomaly. Notice that the septal leaflet of the tricuspid valve hinges distally towards the apex of the right ventricle and the sail-like appearance of the anterior leaflet of the tricuspid valve.
Video 30.8
Ebstein’s anomaly. Parasternal short-axis view looking at the tricuspid valve. Notice that the inflow across the tricuspid valve is also rotated towards the right ventricular outflow tract. There is mild tricuspid regurgitation.
Video 30.9
(a, b) Tetralogy of Fallot. Sub-costal sagital view. Notice the large ventricular septal defect and the anterior deviation of the outlet septum causing right ventricular outflow tract obstruction. (b) Colour Doppler has been added.
Video 30.10
Tetralogy of Fallot. Parasternal long-axis view. Notice large ventricular septal defect extending to the outlet septum with about 40% of aortic override.
Video 30.11
Severe pulmonary regurgitation after Tetralogy of Fallot repair. Parasternal short-axis view. Notice the broad jet of pulmonary regurgitation which starts in the pulmonary artery branches. The right ventricular outflow tract (RVOT) is also dilated, which is related to the wide and dilated right ventricular outflow tract RVOT patch.
Video 30.12
Right ventricular dilatation related to severe pulmonary regurgitation after Tetralogy of Fallot repair. Apical four-chamber view. Notice the dilatation of the right ventricle. Qualitative assessment of right ventricular function suggest preserved systolic function in this patient.
Video 30.13
Patient after the Mustard operation with severe tricuspid regurgitation. Apical four chamber view. Notice the tricuspid regurgitation and mild RV dysfunction.
Video 30.14
Patient after the arterial switch operation. Parasternal short-axis view. Notice the position of the pulmonary artery in front of the aorta after the Lecompte manoeuver. The right pulmonary artery is to the right and the left pulmonary artery to the left of the aorta.
Video 30.15
Congenitally corrected transposition of the great arteries. Apical four-chamber view. In this view, the morphological right ventricle is on the left (notice the septal attachments of the tricuspid valve, the moderator band, and the coarse trabeculations).
Video 30.16
Congenitally corrected transposition of the great arteries. Parasternal short-axis view with the aorta located anterior and to the left of the pulmonary artery. The pulmonary artery bifurcation can be noted.
Video 30.17
Double inlet left ventricle. Apical fourchamber view. Two separate AV-valves are connected to a single ventricle which has left ventricular morphology. Notice the presence of the extra-cardiac Fontan conduit.
Video 30.18
Bidirectional Glenn shunt. Supra-sternal view. Look at the connection between the right superior vena cava and the right pulmonary artery. There is laminar flow on the connection. Flow can also be seen in the proximal left pulmonary artery.
Video 30.19
Total cavopulmonary connection. Supra-sternal view. The bidirectional Glenn shunt can be seen and also the connection between the extra-cardiac conduit and the pulmonary artery (the red flow moving into the pulmonary artery).
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Tay, E., Friedberg, M., Gatzcollis, M.A., Mertens, L. (2010). The Role of Echocardiography in Adult Congenital Heart Disease. In: Zamorano, J.L., Bax, J.J., Rademakers, F.E., Knuuti, J. (eds) The ESC Textbook of Cardiovascular Imaging. Springer, London. https://doi.org/10.1007/978-1-84882-421-8_30
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DOI: https://doi.org/10.1007/978-1-84882-421-8_30
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