Implantable Cardioverter-Defibrillators in Children and Adolescents With Brugada Syndrome.
(2018) Journal of the american college of cardiology : JACC — Vol. 71, n° 2, p. 148-157 (2018)
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- BACKGROUND: Young patients presenting with symptomatic Brugada syndrome have very high risks for ventricular arrhythmias and should be carefully considered for implantable cardioverter-defibrillator (ICD) placement. However, this therapy is associated with high rates of inappropriate shocks and device-related complications. OBJECTIVES: This study investigated clinical features, management, and long-term follow-up of young patients with Brugada syndrome and ICD. METHODS: Patients diagnosed with Brugada syndrome, who underwent implantation of an ICD at an age of ≤20 years, were studied. RESULTS: The study included 35 consecutive patients. The mean age at ICD placement was 13.9 ± 6.2 years. Ninety-two percent were symptomatic; 29% presented with aborted sudden cardiac death and 63% with syncope. During a mean follow-up period of 88 months, sustained ventricular arrhythmias were treated by the ICD in 9 patients (26%), including shocks in 8 patients (23%) and antitachycardia pacing in 1 patient (3%). Three patients (9%) died in an electrical storm. Seven patients (20%) experienced inappropriate shocks, and 5 patients (14%) had device-related complications. Aborted sudden cardiac death and spontaneous type I electrocardiogram were identified as independent predictors of appropriate shock occurrence. CONCLUSIONS: ICD therapy is an effective strategy in young patients with symptomatic Brugada syndrome, treating potentially lethal arrhythmias in >25% of patients during follow-up. Appropriate shocks were significantly associated with previously aborted sudden cardiac death and spontaneous type I electrocardiograms. However, ICDs are frequently associated with complications and inappropriate shocks, both of which remain high regardless of careful device implantation and programming.
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Gonzalez, M. C., Sieira, J., Pappaert, G., de Asmundis, C., Chierchia, G. B., La Meir, M., Sarkozy, A., & Brugada, P. (2018). Implantable Cardioverter-Defibrillators in Children and Adolescents With Brugada Syndrome. Journal of the american college of cardiology : JACC, 71(2), 148-157. https://doi.org/10.1016/j.jacc.2017.10.082 (Original work published 2018)