Abstract

Ventricular tachycardia in Ebstein's anomaly patients is rare and increases the risk of sudden death up to 6 times. We present two cases of Ebstein's anomaly, the first of them with a history of Glenn surgery and biological prosthesis at tricuspid position and the second case without surgical repair. Both admitted to the emergency room due to ventricular tachycardia poorly tolerated. Detailed substrate characterization and pace mapping showed fragmented signals in the area of atrialized right ventricle and correlated were pace mapping reproduced morphology identical to the clinical VT, ablation at this sites were successful without recurrence during follow-up.

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