Catecholaminergic polymorphic ventricular tachycardia with associated sinus node dysfunction.

An 11-year-old Nepalese male child presented with history of recurrent abrupt episodes of syncope for the last one year. There was no family history of sudden death at a young age in his family. ECG at base-line revealed an isorhythmic AV dissociation with a heart rate of 50 bpm and a normal QTc. Echocardiography of the heart was normal. His 24 hour holter study revealed frequent VPC's and episodes of polymorphic ventricular tachycardia. Exercise stress test provoked a polymorphic VT. On EP study, sinus node recovery time (SNRT) was prolonged and ventricular tachycardia (VT) was induced on Isoproterenol infusion. He was treated with a permanent pacemaker and beta-blocker.