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Efficacy of Ventricular Pacing in the Treatment of an Arrhythmic Storm Associated with a Congenital Long QT Mutation |
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| Authors: | Peter F. Aziz MD Maully J. Shah MBBS |
| Affiliation: | 1. Department of Pediatric Cardiology, Cleveland Clinic Lerner College of Medicine, The Cleveland Clinic Foundation, , Cleveland, Ohio, USA;2. The Children's Hospital of Philadelphia Division of Cardiology and the University of Pennsylvania School of Medicine, , Philadelphia, Pa, USA |
| Abstract: | Long QT syndrome in the infant with 2:1 atrioventricular block is a malignant form of disease associated with frequent torsade de pointes in some cases. Those patients that do not respond to antiarrhythmic therapy are particularly challenging to manage. Ventricular pacing in this patient population has been shown to reduce arrhythmic events. We report a case of a newborn with frequent torsade de pointes requiring defibrillation and cardiopulmonary resuscitation with immediate shortening of the QTc interval with ventricular pacing and subsequent resolution of torsade de pointes. |
| Keywords: | Long QT Syndrome (LQTS) Atrioventricular Block (AVB) Torsade de Pointes (TdP) Ventricular Pacing |