Pathophysiology

  Septal tricuspid leaflet is displaced to the apex resulting in atrialization of some part of the right ventricle.

  Tricuspid regurgitation contributes further to the dilatation of right atrium.

  Severe tricuspid regurgitation causes right ventricular volume overload.

  Almost half of the patients have accompanying
Patent Foramen Ovale (PFO) or Atrial Septal Defect (ASD) .



ECG in Ebstein's anomaly

  Right atrial abnormality (RAA). The amplitude of P wave is very high; it may be even higher than the following QRS complex.

  Right bundle branch block (RBBB).

  Low voltage.

  Wolff-Parkinson-White (WPW) syndrome: short PR interval and delta wave (%25).

  Observation of
WPW pattern, RBBB and low voltage together strongly suggests the diagnosis of Ebstein's anomaly.

  Prolongation of PR interval (first degree AV block).

  Deep Q waves in leads II and III.

  QR or Qr complexes in leads C1 to C3.

  Nonspecific T wave changes in precordial leads.

  Atrial flutter or atrial fibrillation.

  Ventricular arrhythmias.




References

  Chou's Electrocardiography in Clinical Practice. Adult and Pediatric. 5th ed. Philadelphia. WB Saunders. 2001.

  Br Heart J 1968;30:617.

  Am J Cardiol 1958;2:210.

  J Am Coll Cardiol 1985;6:1351.

  Am Heart J 1967;73:395.

  Am J Cardiol 197229:826.





ECG 1. The ECG above belongs to a patient with Ebstein's anomaly and atrial septal defect.
The recording was made at a paper speed of 50mm/sec.

Dr. Peter Kukla has donated the above ECG to our website.

Click here for a more detailed ECG





ECG 2. The ECG above belongs to a 16 years old boy who had been operated for Ebstein's anomaly 8 years ago. He had
undergone tricuspid valve replacement. His recent echocardiography showed normal function of the prosthetic valve.

Pediatric Cardiologist Dr. Mahmut Gokdemir has donated the above ECG to our website.

Click here for a more detailed ECG





ECG 3. The ECG above belongs to a 4 days-old newborn with severe Ebstein's anomaly and ASD (atrial septal defect).
Right bundle branch block pattern is seen.

Pediatric Cardiologist Dr. Mahmut Gokdemir has donated the above ECG to our website.

Click here for a more detailed ECG