Abstract 18911: Electrocardiographic Assessment of Brugada Syndrome in Children
Background: Increasing numbers of children are being referred for assessment of Brugada syndrome (BrS) following the diagnosis of BrS in a parent. We sought to study the ECG phenotype of Brugada syndrome in children referred for assessment.
Methods: Subjects were children with a diagnosis of BrS or its mutations, and referred children of adults with a diagnosis of BrS. All standard electrocardiograms (ECGs), modified Brugada lead ECGs, signal-averaged ECGs (SAECGs), and procainamide challenge results were derived from GE Medical ECG systems (MUSE measurement matrix), and compared to a cohort of normal children aged 5 to 15 years.
Results: 41 subjects were evaluated. For standard ECG parameters, subjects referred for assessment of BrS more frequently had Q waves absent in V6 and present in AVR. R-wave amplitudes were lower across the precordial leads, and R wave duration was longer in AVR, but with no R’ wave. No differences were found in ST J-point, midpoint or endpoint levels between subjects and controls in leads V1, V2 or V3. Brugada lead ECGs, with leads V1, V2 or V3 placed one or two interspaces higher, were performed in 36 subjects and identified a higher mean J-point elevation, but control data for comparison was unavailable. SAECGs were abnormal in 20/41 subjects, providing the highest yield of abnormalities and approaching the anticipated 50% estimate of diseased patients among progeny of Brugada parents (Table 1). Procainamide challenge was performed in 8 individuals, but with poor yield.
Conclusion: Children at risk for Brugada syndrome demonstrate differing ECG patterns from normal, but rarely demonstrate ST changes of Brugada type 1 on standard ECG or with procainamide challenge. Signal averaged ECG appears to be the most sensitive test for Brugada syndrome in children, approaching the expected 50% yield anticipated among children of Brugada syndrome parents referred for evaluation.
Author Disclosures: M. Fatah: None. J. Klemensberg: None. S. Sreetharan: None. L. Fenwick: None. M. Lafreniere-Roula: None. R. Hamilton: None.
- © 2016 by American Heart Association, Inc.