OBJECTIVES: 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). METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
OBJECTIVES: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). METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
Authors: Juan Villafane; Jacob R Miller; Julie Glickstein; Jonathan N Johnson; Jonathan Wagner; Chris S Snyder; Tatiana Filina; Scott L Pomeroy; S Kristen Sexson-Tejtel; Caitlin Haxel; Jason Gottlieb; Pirooz Eghtesady; Devyani Chowdhury Journal: Pediatr Cardiol Date: 2021-01-02 Impact factor: 1.655
Authors: Roberta Pereira; Oscar Campuzano; Georgia Sarquella-Brugada; Sergi Cesar; Anna Iglesias; Josep Brugada; Fernando E S Cruz Filho; Ramon Brugada Journal: Clin Res Cardiol Date: 2017-03-16 Impact factor: 5.460
Authors: Roddy Walsh; Arnon Adler; Ahmad S Amin; Emanuela Abiusi; Melanie Care; Hennie Bikker; Simona Amenta; Harriet Feilotter; Eline A Nannenberg; Francesco Mazzarotto; Valentina Trevisan; John Garcia; Ray E Hershberger; Marco V Perez; Amy C Sturm; James S Ware; Wojciech Zareba; Valeria Novelli; Arthur A M Wilde; Michael H Gollob Journal: Eur Heart J Date: 2022-04-14 Impact factor: 35.855
Authors: Georgia Sarquella-Brugada; Oscar Campuzano; Anna Iglesias; Josefina Grueso; David J Bradley; Gunter Kerst; Daniel Shmorhun; Josep Brugada; Ramon Brugada Journal: HeartRhythm Case Rep Date: 2015-05-19