Ventricular tachycardia an atypical presentation of Ebstein’s disease

Ebstein's anomaly (EA) is a rare congenital malformation, characterized by an apical displacement and dysplasia of the septal leaflet of the tricuspid valve with a right ventricular atrialization compromising its function. This malformation includes electrical conduction abnormalities, and very rarely ventricular arrhythmias. We report the case of a 22-year-old male, presenting with dizziness and palpitations. The electrocardiogram showed a sustained monomorphic ventricular tachycardia (SMVT). The presentation of the disease with ventricular tachycardia associated with hemodynamic instability is extremely rare. We placed a surgical implantable cardiac defibrillator when the diagnosis of EA, SMVT, and right ventricular systolic impairment was confirmed.