Abstract
Ebstein’s anomaly is a rare cardiac malformation characterized by varying degrees of dysplasia, apical displacement and insufficiency of the tricuspid valve associated with variable clinical presentation ranging from severely compromised newborns to patients who are asymptomatic into late adulthood. Displacement and dysplasia of the septal leaflet are displayed by 2D echocardiography in the apical four-chamber view and in the parasternal short-axis view, while displacement of the posterior leaflet is visualized in the parasternal long axis of the right ventricular inflow and in the subcostal views. Colour Doppler displays and quantifies tricuspid regurgitation, verifies antegrade flow across the pulmonary valve, determines patency of the ductus arteriosus and reveals direction of shunting across the atrial septum. PW and CW Doppler interrogation of tricuspid regurgitation allows noninvasive assessment of the right ventricular pressure.
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10.1 Electronic Supplementary Material
Video 10.1
The apical four-chamber view in a neonate with Ebstein’s malformation shows severe displacement of the tricuspid valve from the plane of the atrioventricular junction. The plane of the effective tricuspid valve orifice is not displayed in this view. A central line is floating in the right atrium, and the atrial septum with an atrial septal aneurysm is bulging into the left atrium (AVI 24197 kb)
Video 10.2
Colour Doppler in the apical four-chamber view shows inflow across the tricuspid valve (same patient as in Video 10.1). Tricuspid regurgitation is not apparent in this view but cannot be assessed with certainty, since the tricuspid valve orifice is not displayed due to its apical displacement (AVI 7368 kb)
Video 10.3
In this newborn the apical four-chamber view shows adherence and apical displacement of the severely dysplastic septal tricuspid valve leaflet. The anterior leaflet is large and restricted in its mobility due to mural attachments. Both right atrium and right ventricle are enlarged (AVI 31305 kb)
Video 10.4
Colour Doppler in the apical four-chamber view (same patient as in Videos 10.3 and 10.5) reveals severe tricuspid regurgitation (AVI 3417 kb)
Video 10.5
The sweep in the parasternal long-axis view (same patient as in Videos 10.3, 10.4 and 10.6) shows severe dilatation of the right ventricle in the plane of the left ventricular outflow tract. Rightward tilt of the transducer to the parasternal long-axis view of the right ventricular inflow shows significant displacement of the posterior leaflet of the tricuspid valve from the atrioventricular junction and the coronary sinus towards the apex of the right ventricle (AVI 97326 kb)
Video 10.6
Significant displacement of the posterior leaflet of the tricuspid valve is well recognized in the parasternal long-axis view of the right ventricular inflow (same patient as in Videos 10.3, 10.4, 10.5 and 10.7). This is the most important plane to assess the posterior leaflet of the tricuspid valve (AVI 26686 kb)
Video 10.7
Colour Doppler in the parasternal long-axis view of the right ventricular inflow (same patient as in Videos 10.3, 10.4, 10.5 and 10.6) confirms apical displacement of the effective tricuspid valve orifice and reveals moderate tricuspid valve regurgitation (AVI 5657 kb)
Video 10.8
Significant apical displacement of the posterior tricuspid valve leaflet from the tricuspid annulus is apparent in the subcostal coronal view in a neonate with severe Ebstein’s anomaly (WMV 1172 kb)
Video 10.9
Significant displacement is also apparent in the subcostal RAO view (same patient as in Video 10.8). This plan also shows the large anterior tricuspid valve leaflet (WMV 8447 kb)
Video 10.10
The parasternal short-axis view in this neonate with Ebstein’s anomaly shows a large superior tricuspid valve leaflet with attachments to the anterior wall of the right ventricle (AVI 9281 kb)
Video 10.11
Colour Doppler in the parasternal short-axis view (same patient as in Video 10.10) shows unobstructed flow from the right ventricle to the pulmonary artery despite diastolic protrusion of the anterior tricuspid valve leaflet into the right ventricular outflow tract. Note diastolic inflow into the pulmonary artery via a small restrictive patent ductus arteriosus (AVI 2283 kb)
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Hofbeck, M., Deeg, KH., Rupprecht, T. (2017). Ebstein’s Anomaly. In: Doppler Echocardiography in Infancy and Childhood. Springer, Cham. https://doi.org/10.1007/978-3-319-42919-9_10
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DOI: https://doi.org/10.1007/978-3-319-42919-9_10
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