Efficacy of ventricular pacing in the treatment of an arrhythmic storm associated with a congenital long QT mutation.
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.
Department of Pediatric Cardiology, Cleveland Clinic Lerner College of Medicine, The Cleveland Clinic Foundation, Cleveland, Ohio, USA.
Peter F
PF
Aziz
Maully J
MJ
Shah
D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D054537:Atrioventricular Block / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology / Q000628:therapy*; D002304:Cardiac Pacing, Artificial / Q000379:methods*; D004252:DNA Mutational Analysis; D000072237:ERG1 Potassium Channel; D004562:Electrocardiography; D051638:Ether-A-Go-Go Potassium Channels / Q000235:genetics; D020022:Genetic Predisposition to Disease; D006339:Heart Rate; D006801:Humans; D007231:Infant, Newborn; D051657:KCNQ1 Potassium Channel / Q000235:genetics; D008133:Long QT Syndrome / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology / Q000628:therapy*; D009154:Mutation; D010375:Pedigree; D010641:Phenotype; D016171:Torsades de Pointes / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology / Q000628:therapy*; D016896:Treatment Outcome; D016276:Ventricular Function
D000889:Anti-Arrhythmia Agents; D000072237:ERG1 Potassium Channel; D051638:Ether-A-Go-Go Potassium Channels; D051657:KCNQ1 Potassium Channel; C496909:KCNQ1 protein, human