Assessing Ebstein's anomaly

Ebstein's anomaly isn't just a valve problem. In this video, from our Echo Masterclass: Adult Congenital Heart Disease course, you'll discover the cause of Ebstein's anomaly, the commonly associated lesions, and which cardiac elements are involved.

Flustered at the thought of performing an adult congenital echo? Don’t be! In our Echo Masterclass: Adult Congenital Heart Disease course, you’ll learn about the lesions associated with common types of defects, as well as the common surgical repair complications. Explore the hemodynamics of intracardiac defects, master key assessment strategies (such as the sequential segmental approach), and know what to leave out of the echo report.

[00:00:00] Ebstein anomaly is primarily characterized by the AP Corti displacement of the tricuspid valve leaflets. The anterior leaflet also has abnormal and thickened Corti and the valve also rotates anteriorly closer towards the pulmonary valve. Assessment of the valve is obviously pretty important here. But what else do we need to know? First of all, Ebstein anomaly is strongly associated with atrial septal defects, which can be an important cause of cyanosis. So it is important to assess the atrial septum very carefully. This patient with Ebstein's has an atrial

[00:00:30] septal defect with bidirectional flow. It will flow right to left in systole when the right atrium fills with tricuspid regurgitation, and then in diastole will flow left to right as both atria fill with venous blood. Secondly, let's take another look at this image. This time concentrate on the

[00:01:00] size of the chambers. The displacement of the valve causes a lot of the right ventricle to become atrialized, or to function like a right atrium. All that atrial stretch can lead to arrhythmia. But how does all of that affect the right ventricle? Well, it actually looks pretty small, maybe it's just a cut. Let's take a look in the short axis view. Ouch. It looks pretty small here too. Sometimes they might be right ventricular outflow obstruction due to the Corti obstructing the small

[00:01:30] right ventricular cavity. This is associated with very early childhood presentation sometimes in utero and carries a poor prognosis. So particularly if there is tricuspid regurgitation, the little right ventricle has an awful lot of work to do, right? That's right. Take a look at this case. Here we are looking at a severe form of Ebstein. In the four-chamber view, it's very difficult to see the right ventricle at all. This patient has severe tricuspid regurgitation so the right atrium

[00:02:00] is really overloaded. Here's the short access view. The tricuspid annulus is almost touching the pulmonary valve. The right ventricle is so small but it seems to be very dynamic. It's small but powerful. This patient had some other tests and was found to be desaturating with exercise despite a very limited exercise capacity. So they would take into surgery to have the valve replaced. The patient had a bioprosthetic valve placed in the normal location for a tricuspid valve.

[00:02:30] Now the valve does a much better job, almost no regurgitation at all. Let's look at the right ventricle then. Well, it's definitely bigger. That should be a good thing. But how do you think it works? It's not contracting very well at all. The right ventricle of this patient from this example has never really improved and the patient has ongoing right heart failure despite the valve being replaced. The right ventricle and Ebstein anomaly frequently has

[00:03:00] abnormal myocardial development. This case shows that the Ebstein anomaly isn't just a disease at the valve but that the ventricle is involved too. Here's another patient with Ebstein who was sent for valve replacement. There's a bit more right ventricle to work with in this case. After a successful valve replacement at the normal level of the annulus, the right ventricle is bigger here too. The ventricular function also looks pretty good but look at the base of the ventricular septum. This area used to be

[00:03:30] atrialized and is now functioning at ventricular pressure. The basal septum motion is quite abnormal and this is typical following valve replacement in these patients. So assessment of the valve is obviously a priority in Ebstein patients. Atrial septal defects are commonly associated and abnormal development of the right ventricle necessitates careful assessment both before and after surgery.