Short QT Syndrome Manifesting with Neonatal Atrial Fibrillation and Bradycardia

<b><i>Objectives:</i></b> Atrial fibrillation (AF) is rare during childhood and usually associated with other cardiovascular pathology. In lone AF, the ventricular response rate is usually rapid. We sought to describe a subset of children who present with early-onset AF and a slow ventricular response rate who were found to have the short QT syndrome (SQTS). <b><i>Methods:</i></b> Using a MEDLINE/PubMed search, children with AF, a structurally normal heart and bradycardia were identified. Demographics, clinical presentation, electrocardiographic (ECG) findings, electrophysiologic testing, genetic analysis and follow-up assessment were collected on each child for analysis. <b><i>Results:</i></b> Four children were identified in the literature and combined with 2 other children followed by the authors. All had a short QT interval and those who were tested were found to have a gain-of-function mutation in the KCNQ1 gene. <b><i>Conclusions:</i></b> We describe a subclass of children with SQTS who present with AF and a slow ventricular response. Medical therapy has not been effective in maint