Abstract
Ebstein's anomaly, a rare and highly variable congenital heart defect, still presents a treatment challenge. The currently used cone repair of the tricuspid valve has carried favorable results in suitable patients.
Arrhythmogenic substrates including accessory pathways and right bundle branch block associated with electromechanical ventricular dyssynchrony present additional therapeutic targets. We present a patient with Ebstein's anomaly of tricuspid valve and Wolff-Parkinson-White syndrome in whom joint electrophysiological and surgical interventions were used to address all major disease components.
This report is unique in highlighting a combination of different treatment modalities for managing severe Ebstein's anomaly, including catheter pathway mapping and ablation, surgery with cryoablation, and right ventricular cardiac resynchronization therapy (RV-CRT), with optimal results.