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|Number 11-22: Ebstein Anomaly in an Asymptomatic Air Force Professional|
Number 11-22: Ebstein Anomaly in an Asymptomatic Air Force Professional
Case from: Javed M. Nasir1, Andrew Crean2, Kevin Steel1
Chest roentgenography was normal.
She then underwent transthoracic echocardiography which showed mild TR, Ebstein anomaly, and a weakly positive bubble study. There was no evidence of right ventricular outflow obstruction, and subsequent transesophageal echocardiogram showed no atrial or ventricular septal defects.
TTE 4 chamber TTE 4 chamber with color
4chamber CMR with TR
After diagnosing Ebstein anomaly in this active duty airwoman, she was returned to her prior duties with no limitations on her career or physical training, other than following up annually with a cardiologist.
4 chamber CMR static with measurement
Ebstein anomaly is a rare congenital disorder that occurs in approximately 1 out of every 200,000 live births and accounts for <1% of congenital heart disease.1 This disease is characterized by an apical displacement of the tricuspid leaflets (septal>posterior>anterior). This anomaly causes an atrialization of the right ventricle and can lead to cyanosis, tricuspid regurgitation, and right ventricular outflow tract (RVOT) obstruction or functional tricuspid stenosis. Ebstein is associated with many other cardiac defects and more than half of patients with Ebstein anomaly have atrial shunts, 25% have an accessory conduction pathway, and there is also an increased risk of ventricular septal defects and varying degrees of RVOT obstruction.2
While the overall prognosis for Ebstein anomaly is poor, most of the natural history trials were published prior to use of echocardiography and mainly included severe disease that was diagnosed as an infant. More recent data demonstrates that when Ebstein anomaly is initially diagnosed in adulthood, there are less associated cardiac defects, cyanosis is rare, and it is associated with a much lower mortality and morbidity.6
Perspective: Ebstein anomaly remains a rare type of congenital heart disease with many associated cardiac defects. As this case illustrates, Ebstein anomaly can be subtle on the MRI and it is important to routinely measure the tricuspid septal leaflet apical displacement and normalize for BSA to ensure mild cases of Ebstein anomaly are not missed.
Do you want to comment on the breast implants of the patient and the possible effects on ECG findings (decrease in voltage)? When were the breast implants implanted? Before or after the ablation in 2006?
Note: The breast implants were implanted four years prior to the ablation and may have masked some of the voltage from her pathway. However, no pre ablation ECG is available.
1. Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein's Anomaly. Circulation. 2007;115:277-285.
2. Warnes CA, Williams RG, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). J Am Coll Cardiol. 2008 Dec 2;52(23):e1-121.
3. Gussenhoven EJ, Stewart PA, Becker AE, Essed CE, Ligtvoet KM, De Villeneuve VH "Offsetting" of the septal tricuspid leaflet in normal hearts and in hearts with Ebstein's anomaly. Anatomic and echographic correlation. Am J Cardiol. 1984;54(1):172-6.
4. Shiina A, Seward JB, Edwards WD, Hagler DJ, Tajik AJ. Two-dimensional echocardiographic spectrum of Ebstein's anomaly: detailed anatomic assessment. J Am Coll Cardiol. 1984;3(2 Pt 1):356.
5. Yalonetsky S, Tobler D, Greutmann M, Crean AM, Wintersperger BJ, Nguyen ET, Oechslin EN, Silversides CK, Wald RM. Cardiac magnetic resonance imaging and the assessment of ebstein anomaly in adults. Am J Cardiol. 2011 Mar 1;107(5):767-73. Epub 2011 Jan 19.
6. Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID, Allan L, Nihoyannopoulos P, Somerville J, Deanfield JE. Ebstein's anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol. 1994;23(1):170.
Have your say: What do you think? Latest posts on this topic from the forum
Re: Number 11-22: Ebstein anomaly
Yes, Ebsteins anomaly may be mild and patients may go through life undiagnosed. And yes, those who end up having a CMR study are those with more severe disease. However, as a congenital Cardiologist, and having dealt with the spectrum...
On: 02/13/2012 By: Henrimatahari Read more?
Re: Number 11-22: Ebstein anomalyrdiomyopathy
Really educative case! As a cardiovascular radiologist not doing echo, the fact that ebstein anomaly in varying degrees is not uncommon to see in cardiology practice was a revelation. We usually tend to see obvious ebstein on cmr ,when cases...
On: 02/11/2012 By: GULATIGURPREET Read more?
Re: Number 11-22: Ebstein anomaly
Excellent case including the interesting footnote. I think it is a good point to routinely measure the septal tricuspid valve leaflet and mitral annular distance to pick up clinically mild form of Ebstein's anomaly to know the true prevalance.Interestingly...
On: 11/20/2011 By: ansumansaha Read more?
Number 11-22: Ebstein anomaly
Very interestering new case this week. How common is to diagnose Ebstein as an incidental finding? ...
On: 11/16/2011 By: chiarabd Read more?