Skip to Main Content




A 32-year-old woman presented for evaluation of decreasing exercise tolerance and a loud heart murmur. On physical examination, she had a fixed split second heart sound with a normal pulmonary component. She had a holosystolic murmur at the mid-left sternal border that did not radiate and increased with inspiration. An electrocardiogram (ECG) and echocardiogram were performed. ECG demonstrated right atrial enlargement and a right bundle branch block. Echocardiogram showed apical displacement of the septal and posterior leaflets of her tricuspid valve resulting in severe tricuspid regurgitation. Her clinical findings were consistent with Ebstein's anomaly. Further testing included a functional vo2 exercise stress test. With exercise, she was found to have significant oxygen desaturation to the mid-80s on room air. A subsequent transesophageal echocardiogram showed a patent foramen ovale (PFO) with bidirectional shunting, severe tricuspid regurgitation, and an apically displaced septal leaflet of the tricuspid valve consistent with Ebstein's anomaly. Because of her significant hypoxia with exercise, she underwent a catheter-based closure of her PFO. A repeat exercise study following PFO closure showed normal oxygen saturations with exercise and improved exercise capacity. She was followed with serial imaging that continued to demonstrate the severe tricuspid regurgitation and gradual worsening in her functional capacity. She developed atrial flutter with rapid ventricular rate for which she needed to be cardioverted. She was managed with antiarrhythmic medications and has had no further arrhythmia recurrences. She finally underwent surgical management in the form of a tricuspid valve replacement with a bioprosthetic valve and plication of the atrialized ventricle. The patient had an uneventful postoperative recovery with significant improvement in her functional capacity.




  • This case highlights several important concepts for the long-term complications and management of adult patients with Ebstein's anomaly of the tricuspid valve.

  • These include the concerns for atrial arrhythmias, atrial level shunting secondary to an interatrial communication, severity of tricuspid regurgitation, and declining functional capacity.

  • Ebstein's anomaly is a rare congenital heart disorder (1/200,000 live births), accounting for about 0.3% to 0.7% of all cases of congenital heart disease (CHD), however this case highlights that Ebstein's anomaly can present later in life since patients can have a wide spectrum of symptoms that may prevent the underlying pathology from being discovered until adulthood.

  • Such patients require regular long-term cardiac follow-up with an adult congenital cardiologist to prevent long-term cardiac complications.




  • Ebstein's anomaly is a rare congenital heart disease that consists of apical displacement of the septal leaflet of the tricuspid valve and atrialization of the right ventricle. The estimated risk of Ebstein's anomaly in the general population is 1 in 200,000 live births, with males and females being at equal risk.1

  • Lithium use during pregnancy has been considered a risk factor for developing Ebstein in the fetus, although the classic case series of "Lithium babies" reported by Weinstein and Goldfield in 1975 showed an extremely high ...

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.


About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Subscription Options

AccessCardiology Full Site: One-Year Subscription

Connect to the full suite of AccessCardiology content and resources including textbooks such as Hurst's the Heart and Cardiology Clinical Questions, a unique library of multimedia, including heart imaging, an integrated drug database, and more.

$595 USD
Buy Now

Pay Per View: Timed Access to all of AccessCardiology

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.