We report a case with Ebstein's anomaly and pulmonary atresia with sustained monomorphic ventricular tachycardia in a patient without a ventriculotomy history. In the low voltage area between the atrialised right ventricle and hypoplastic right ventricle, there was a ventricular tachycardia substrate and slow conduction. The tachycardia circuit was eliminated by a point catheter ablation at the area with diastolic fractionated potentials.
Keywords: Ebstein’s anomaly; Point ablation; total cavopulmonary connection; ventricular tachycardia.