AUTHOR=Sarquella-Brugada Georgia , García-Algar Oscar , Zambrano María Dolores , Fernández-Falgueres Anna , Sailer Sebastian , Cesar Sergi , Sebastiani Giorgia , Martí-Almor Julio , Aurensanz Esther , Cruzalegui Jose Carlos , Merchan Erika Fernanda , Coll Mónica , Pérez-Serra Alexandra , Olmo Bernat del , Fiol Victoria , Iglesias Anna , Ferrer-Costa Carles , Puigmulé Marta , Lopez Laura , Pico Ferran , Arbelo Elena , Jordà Paloma , Brugada Josep , Brugada Ramon , Campuzano Oscar
TITLE=Early Identification of Prolonged QT Interval for Prevention of Sudden Infant Death
JOURNAL=Frontiers in Pediatrics
VOLUME=9
YEAR=2021
URL=https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2021.704580
DOI=10.3389/fped.2021.704580
ISSN=2296-2360
ABSTRACT=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.