A 10-year-old girl developed life-threatening recurrent polymorphic ventricular tachycardia following surgical closure of a simple secundum atrial septal defect. Post hoc analysis of a Holter recording suggested Brugada syndrome. After managing the acute phase, a dual chamber defibrillator was implanted. One week later she experienced VF storm, needing 96 appropriate shocks within a few hours. Quinidine, by virtue of its I(to) blocking property, is the only drug reported to be useful in managing VF storm in Brugada syndrome. Nonavailability of quinidine led us to try its diastereomer, intravenous quinine, which succeeded in controlling the ventricular tachycardia. Arrhythmia storm in the setting of ion channelopathy can be difficult to manage, and sometimes requires innovative therapies.
© 2010 Wiley Periodicals, Inc.