Prolonged Q-T interval syndrome. Successful treatment with combined ventricular pacing and propanolol

A case of the sporadic variety of the prolonged Q-T interval syndrome without deafness is presented. The patient is unusual because of a long survival to age 13, despite progressive worsening of her disease, and the presence of marked atrioventricular conduction abnormalities in addition to the usual ventricular trachyarrhythmias. Successful therapy was finally achieved with a combination of permanent pervenous ventricular pacing and propranolol.