PubMed 24818999

Referenced in Channelpedia wiki pages of: none

Automatically associated channels: KCNQ1 , Kv7.1 , Slo1

Title: Short QT syndrome manifesting with neonatal atrial fibrillation and bradycardia.

Authors: Juan Villafane, Peter Fischbach, Roman Gebauer

Journal, date & volume: Cardiology, 2014 , 128, 236-40

PubMed link:

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).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.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.We describe a subclass of children with SQTS who present with AF and a slow ventricular response. Medical therapy has not been effective in maintaining sinus rhythm. The long-term outcome remains unknown for these children. This condition may present in utero as persistent bradycardia with postnatal ECG showing a very short QT interval.