July 17, 2026 · European heart journal · DOI: 10.1093/eurheartj/ehag513

Paediatric long QT syndrome: clinical outcomes and therapy in the Spanish National Registry

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This study aimed to characterize the clinical features, management strategies, and predictors of major arrhythmic events (MAEs) in a nationwide cohort of children diagnosed with congenital long QT syndrome (LQTS) in Spain. The findings revealed that MAEs were rare, primarily occurring in children with high-risk genotypes and significantly prolonged QTc intervals, supporting a genotype and QTc-guided management approach with beta-blockers as the primary treatment. Additionally, the study highlighted the association of foetal/neonatal bradycardia with adverse outcomes, emphasizing the need for careful monitoring in this population.

Francesca Perin, Antonio J Cartón, Francisco Jose Bermúdez-Jiménez, Bárbara C Fernández-Barrio, Roger Esmel-Vilomara, Laura Marcos-Fuentes, Fernando Rueda Nuñez, Maria Del Mar Rodríguez Vázquez Del Rey, Elena Montañés Delmás, Ariadna Ayerza-Casas, Belén Fernández-Tudela, Maria Teresa Viadero, Francisco Castro, Ana Siles, Enrique Blanca-Jover, Laura Caravaca-Pantoja, Andrea Bueno-Gómez, Beatriz Salamanca-Zarzuela, Paula De Vera McMullan, Gloria López Blanco, Beatriz Plata-Izquierdo, Soledad Jimenez Casso, Maria Isabel Serrano Robles, Miguel Ángel Matamala Morillo, Laura Del Rey Megias, Sandra Rocamora Salort, Carlota Aparicio Fernández de Gatta, Marta Villares Alonso, Maria Arroyas Sánchez, Angeles Ortega Montes, Andrea Greco, Estefanía Martínez-Barrios, Juan Jiménez-Jáimez, Ferran Roses-Noguer, Georgia Sarquella-Brugada

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Paediatric long QT syndrome: clinical outcomes and therapy in the Spanish National Registry

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