What is the incidence of Ebstein anomaly in Wolff-Parkinson-White (WPW) syndrome?

Updated: Jan 08, 2017
  • Author: Christopher R Ellis, MD, FACC, FHRS; Chief Editor: Mikhael F El-Chami, MD  more...
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Approximately 10% of patients with Ebstein anomaly of the tricuspid valve have WPW syndrome. They usually have more than 1 accessory pathway (AP), and those are usually on the right side. Patients with corrected transposition of the great arteries and left-side Ebstein anomaly may also have WPW syndrome. In these patients, the AP is on the left side or septal.

Patients with Ebstein anomaly of the tricuspid valve may present with cyanosis, tachypnea, and other signs of congestive heart failure in presence of a rapid heart rate. ECG may show either wide or narrow QRS, SVT, and, sometimes, QRS with changing morphology if more than one AP is present. Patients with right-side accessory pathways should be screened for the Ebstein anomaly by echocardiography.

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