Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome.
(2019) Journal of the american college of cardiology : JACC — Vol. 73, n° 14, p. 1756-1765 (2019)
Files
2019_Michowitz_J_Am_Coll_Cardiol.pdf
Open Access - Adobe PDF
- 336.69 KB
Details
- Authors
- Abstract
- BACKGROUND: Information on young patients with Brugada syndrome (BrS) and arrhythmic events (AEs) is limited. OBJECTIVES: The purpose of this study was to describe their characteristics and management as well as risk factors for AE recurrence. METHODS: A total of 57 patients (age ≤20 years), all with BrS and AEs, were divided into pediatric (age ≤12 years; n = 26) and adolescents (age 13 to 20 years; n = 31). RESULTS: Patients' median age at time of first AE was 14 years, with a majority of males (74%), Caucasians (70%), and probands (79%) who presented as aborted cardiac arrest (84%). A significant proportion of patients (28%) exhibited fever-related AE. Family history of sudden cardiac death (SCD), prior syncope, spontaneous type 1 Brugada electrocardiogram (ECG), inducible ventricular fibrillation at electrophysiological study, and SCN5A mutations were present in 26%, 49%, 65%, 28%, and 58% of patients, respectively. The pediatric group differed from the adolescents, with a greater proportion of females, Caucasians, fever-related AEs, and spontaneous type-1 ECG. During follow-up, 68% of pediatric and 64% of adolescents had recurrent AE, with median time of 9.9 and 27.0 months, respectively. Approximately one-third of recurrent AEs occurred on quinidine therapy, and among the pediatric group, 60% of recurrent AEs were fever-related. Risk factors for recurrent AE included sinus node dysfunction, atrial arrhythmias, intraventricular conduction delay, or large S-wave on ECG lead I in the pediatric group and the presence of SCN5A mutation among adolescents. CONCLUSIONS: Young BrS patients with AE represent a very arrhythmogenic group. Current management after first arrhythmia episode is associated with high recurrence rate. Alternative therapies, besides defibrillator implantation, should be considered.
- Affiliations
Citations
Michowitz, Y., Milman, A., Andorin, A., Sarquella-Brugada, G., Gonzalez, M. C., Gourraud, J.-B., Conte, G., Sacher, F., Juang, J. J. M., Kim, S.-H., Leshem, E., Mabo, P., Postema, P. G., Hochstadt, A., Wijeyeratne, Y. D., Denjoy, I., Giustetto, C., Mizusawa, Y., Huang, Z., et al. (2019). Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome. Journal of the american college of cardiology : JACC, 73(14), 1756-1765. https://doi.org/10.1016/j.jacc.2019.01.048 (Original work published 2019)