Key result
Epicardial mapping reveals unexpected AF in a 14-month-old with short QT syndrome.
Case Report (n=1)
Epicardial mapping identified atrial fibrillation in a very young (14-month-old) patient with short QT syndrome, highlighting a rare presentation.
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Alerts clinicians to possible AF in infants with short QT syndrome; hypothesis-generating and requires validation in larger cohorts.
Short QT syndrome (SQTS) is a very rare channelopathy accompanied with familial clustering and sudden cardiac.1 It has an estimated prevalence ranging from 0.02% up to 2% in the adult population, but only 0.05% among pediatric patients.2-6 Up to today, nine mutations in six different genes have been identified, including KCNH2, KCNQ1, KCNJ1, CACNA1C, CACNB2 and CACNA2D1. In pediatric patients, SQTS is characterized by shortening of the corrected QT interval (QTcB <316 ms, J-Tpeak cB <181 ms and the presence of early repolarization) on the surface electrocardiogram (ECG).
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Schie et al. (2022) conducted a case report in Short QT syndrome (SQTS) with atrial fibrillation (n=1). Epicardial mapping was evaluated. Epicardial mapping revealed an unexpected finding of atrial fibrillation in a 14-month-old patient with short QT syndrome.
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