Syncopal sustained ventricular tachycardia in a patient with right ventricular dysplasia

A 41-year-old man was hospitalized for syncopal sustained ventricular tachycardia with left bundle branch morphology. Diagnostic screening confirmed a right ventricular dysplasia: fibrofatty replacement of myocardium on endomyocardial biopsy and severe dilation of right ventricle with no left ventricular impairment was documented. His bundle recording showed an abnormally long HV interval, and programmed ventricular stimulation induced high-rate sustained ventricular tachycardia with left bundle branch block morphology associated with reduced systolic blood pressure and dizziness. Right ventricular burst pacing proved to be effective in restoring sinus rhythm. A single lead pectoral cardioverter-defibrillator was implanted and programmed for VVI and antitachycardia pacing, as first ventricular tachycardia therapeutic intervention. During 6-month follow-up, 1 asymptomatic ventricular tachycardia recurrence terminated by antitachycardia pacing was reported by the device. The possible role of the other therapeutic options such as drugs, ablation, and surgery for this specific case is also discussed.