Arq. Bras. Cardiol. 2021; 117(1 Supl. 1): 19-22
Life-Threatening Ventricular Arrhythmia Induced by Atrial Tachycardia in a Child with an SCN5A Mutation
Márcio Augusto Silva
, Jorge Elias Neto
, Guilherme Muller de Campos Futuro, Erick Sessa Merçon, Deborah Vasconcelos, Ricardo Kuniyoshi
Introduction
Mutations in the SCN5A gene, encoding the cardiac Na+channel, can result in several life-threatening arrhythmias. These mutations have proven to be causative for inherited and primarily electrical diseases, including Brugada Syndrome (BrS), Long QT Syndrome, and other cardiac conduction disturbances., BrS, the most reported condition in this group of disorders, has typically been described in adult populations and related to approximately 20% of all sudden deaths (SD) in patients with apparently normal hearts.– Therefore, few reports have shown significant arrhythmic events (AE) caused by this mutation in childhood.
Atrial arrhythmias, as well as sick sinus syndrome (SSS), may be related to Na+channel abnormalities. In BrS, atrial arrhythmias are being diagnosed in up to 38% of patients and are related to worse prognosis.
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Keywords: Brugada Syndrome; Cardiac Arrhythmia; Child; Syncope
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