Early Identification of Prolonged QT Interval for Prevention of Sudden Infant Death

Introduction: Long QT syndrome is the main arrhythmogenic disease responsible for sudden death in infants, especially in the first days of life. Performing an electrocardiogram in newborns could enable early diagnosis and adoption of therapeutic measures focused on preventing lethal arrhythmogenic e...

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Detalles Bibliográficos
Autores: Sarquella Brugada, Geòrgia, García-Algar, Oscar, Zambrano, María Dolores, Fernández-Falgueras, Anna, Sailer, Sebastian, Cesar, Sergi, Sebastiani, Giorgia, Martí-Almor, Julio, Aurensanz, Esther, Cruzalegui, José, Merchan, Erika Fernanda, Coll Vidal, Mònica, Perez-Serra, Alexandra, Olmo, Bernat del, Fiol, Victoria, Iglesias, Anna, Ferrer Costa, Carles, Puigmulé, Marta, López López, Laura, Picó, Ferran, Arbelo, Elena, Jordà, Paloma, Brugada Terradellas, Josep, Brugada, Ramon, Campuzano Larrea, Oscar
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2021
País:España
Institución:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
Repositorio:Recercat. Dipósit de la Recerca de Catalunya
OAI Identifier:oai:recercat.cat:10256/20333
Acceso en línea:http://hdl.handle.net/10256/20333
Access Level:acceso abierto
Palabra clave:Infants nadons -- Malalties
Newborn infants -- Diseases
Mort sobtada -- Prevenció
Sudden death -- Prevention
Descripción
Sumario:Introduction: Long QT syndrome is the main arrhythmogenic disease responsible for sudden death in infants, especially in the first days of life. Performing an electrocardiogram in newborns could enable early diagnosis and adoption of therapeutic measures focused on preventing lethal arrhythmogenic events. However, the inclusion of an electrocardiogram in neonatal screening protocols still remains a matter of discussion. To comprehensively analyse the potential clinical value of performing an electrocardiogram and subsequent follow-up in a cohort of newborns. Methods: Electrocardiograms were performed in 685 neonates within the first week of life. One year follow-up was performed if QTc > 450 ms identified. Comprehensive genetic analysis using massive sequencing was performed in all cases with QTc > 470 ms. Results: We identified 54 neonates with QTc > 450 ms/ <470 ms; all normalized QTc values within 6 months. Eight cases had QTc > 480 ms at birth and, if persistent, pharmacological treatment was administrated during follow-up. A rare variant was identified as the potential cause of long QT syndrome in five cases. Three cases showed a family history of sudden arrhythmogenic death. Conclusions: Our prospective study identifies 0.14% of cases with a definite long QT, supporting implementation of electrocardiograms in routine pediatric protocols. It is an effective, simple and non-invasive approach that can help prevent sudden death in neonates and their relatives. Genetic analyses help to unravel the cause of arrhythmogenic disease in diagnosing neonates. Further, clinical assessment and genetic analysis of relatives allowed early identification of family members at risk of arrhythmias helping to adopt preventive personalized measures