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Ebstein-WPW Association — EECC MCQ

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ModerateCongenital Heart Disease (Adult)Ebstein-WPW AssociationEECC

A 28-year-old man with Ebstein anomaly has accessory pathway-mediated tachycardia (WPW). EP study reveals multiple right-sided accessory pathways. What is the association between Ebstein anomaly and WPW?

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Correct answer: D — Ebstein anomaly has a 10-25% prevalence of WPW syndrome — the displaced tricuspid valve annulus creates abnormal connections between atrial and ventricular myocardium; multiple pathways and right-sided pathways are characteristic; ablation success is lower than for typical WPW due to pathway location and multiplicity

Ebstein anomaly has the highest prevalence of WPW syndrome among all congenital heart defects (10-25%). The association results from: (1) apical displacement of the tricuspid valve leaflets creates an abnormal posterior annular region where accessory myocardial connections (bypass tracts) persist from embryological development; (2) pathways are typically RIGHT-SIDED (posteroseptal, posterior, or posterolateral — matching the area of leaflet displacement); (3) MULTIPLE pathways are common (15-30% of Ebstein WPW patients); (4) ablation challenges: (a) abnormal anatomy distorts standard landmarks; (b) multiple pathways require comprehensive mapping; (c) pathways may be broad or epicardial; (d) success rates are lower (~75-85% vs >95% for standard WPW) and recurrence is higher. The 2020 ESC ACHD and 2019 SVT Guidelines recommend ablation at experienced ACHD-EP centres for symptomatic Ebstein-WPW.

Reference: ESC (2020): ACHD; ESC (2019): SVT Guidelines