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Echo-of-the-Day
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Echo-of-the-Day Information
The Echo-of-the-Day is a presentation of 1 or more video loops to present an important topic in echocardiography. Please reveiw the image or video loops and then answer the questions below. After you have answered the questions you can view the explanation and obtain CME credit (if available).

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Preop Preop TEE or TTE
Intraop An Intraop TEE
IntraopPreOp Intraop TEE or TTE before Operation
IntraopPostOp Intraop TEE or TTE after Operation
IntraopEnd Intraop TEE or TTE at End of Anesthesia
Post Op Postop TEE or TTE
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Echo of the Day Information Table
IntraopEnd is usually a TEE after the Operation and after some event occured to show a change in the TEE
Pel and Wilhelm did not have a feverish fight over this diagnosis
Case#: 289
AMA CME Units: 0.25 Units
Estimated Time: 15 minutes.
Presentation:
A patient presented with WPW and these echo loops:
 
Loop(s):
Case Discussion
 
The diagnosis is Epstein's Anomaly (EA).

The echocardiographic signs of EA are

  • TV Annulus in normal position
  • Apical Insertion of the Tricuspid Valve
  • Atrialization of the Right ventricle
  • Enlarged Right Atrium
  • Enlarged TV Anterior Leaflet
  • Small TV Septal and Posterior Leaflet
  • RV Dilation

The symptoms occur from the TR, other associated congenital anomalies and conduction abnormalities that may be present. EA is associated with the following congenital anomalies:

  • ASD/PFO
  • Bicuspid Aortic Valve (BAV)
  • Atretic AV
  • Pulmonary Atresia
  • Hypoplastic PA
  • Subaortic Stenosis
  • Coarctation
  • MV Prolapse
  • LV Moderator Bands
  • VSD
  • Pulmonary Stenosis (PS)
  • LV Noncompaction

50% of patients with EA will have WPW. Other conduction abnormalities are large P waves, 1st degree AV block, RBBB, inferior Q waves and anterior T wave inversion. Patients will typically present with

  • Cyanosis
  • SOB/DOE
  • Palpitations/Arrhythmias
  • Sudden Death
  • TIA/CVA
  • Right Sided Heart Failure

The treatment for EA, given the high mortality rate in neonates, is surgical repair or replacement. Most medical management focuses on arrhythmima prevention and management of the right sided heart failure.




Dr. Wilhelm Epstein discovered this condition and it was named after him (Epstein's Anomaly). Dr. Epstein also founded another condition where patients with Hodgkin's disease had cyclic fevers that increase and then decrease over one or two weeks. The cyclic phonomena was also discovered by Dr. P.K. Pel (who published the condition just before Epstein). Therefore, the condition was called Epstein-Pel fever (interesting that it wasn't called Pel-Epstein fever). They apparently argued over the etiology of the condition. Epstein-Pel fever only occurs in 5-10% of Hodgkin's patients. Dr. Richard Asher of the Medical Society of London, in 1959, felt that this condition only exists because it has a name.

 
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