Implantable Cardioverter-Defibrillators in Children and Adolescents With Brugada Syndrome
Key result
ICD therapy treats sustained arrhythmias in 26% of young Brugada patients despite frequent inappropriate shocks.
- n=35
Why the study?
Young patients with symptomatic Brugada syndrome have high risks for ventricular arrhythmias, but ICD placement is associated with high rates of inappropriate shocks and complications.
Key Points
- To evaluate the clinical outcomes, efficacy, and complication rates associated with implantable cardioverter-defibrillator placement in children and adolescents diagnosed with Brugada syndrome.
- Retrospective evaluation of pediatric and adolescent patients diagnosed with Brugada syndrome who underwent implantable cardioverter-defibrillator (ICD) implantation.
- Assessment of clinical indications, appropriate and inappropriate shock therapy rates, and device-related complications during long-term follow-up.
- Implantable cardioverter-defibrillators effectively terminate life-threatening ventricular tachyarrhythmias in pediatric Brugada syndrome patients.
- High rates of device-related complications and inappropriate shocks occur over extended follow-up, necessitating careful risk stratification.
Study Design
Type
Cohort (n=35)
PICO
P
Population
35 young patients (mean age 13.9 years) with Brugada syndrome who underwent ICD implantation at age ≤20 years, followed for a mean of 88 months.
E
Exposure / Comparator
Implantable cardioverter-defibrillator (ICD)
O
Primary Outcome
Sustained ventricular arrhythmias treated by the ICD
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Key expert perspectives
Captured external expert commentary on this paper, strongest first. Original sources are linked where available.
“These 2 undesirable events occur despite very careful device implantation and programming in experienced hands. It is thus extremely important to evaluate every patient on an individual basis and discuss with the patient and the family the benefits and potential risks associated with this therapy. Long-term follow-up studies are needed to assess the value of diagnostic electrophysiological testing and of specific genetic mutations in this population.”
“Despite the fact that ICD therapy is the only proven intervention to prevent sudden cardiac death, overzealous implantation should be avoided at all costs. In the current state of device technology, even in carefully selected at risk patients as described in this study, the dark side of implantation is unavoidable.”
Implication
Clinical analysis observes outcomes of implantable cardioverter-defibrillators in children with Brugada syndrome, highlighting device efficacy and management considerations.
Abstract
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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Cite This Study
Corcia et al. (2018) conducted a cohort in Brugada syndrome (n=35). Implantable cardioverter-defibrillator (ICD) was evaluated on Sustained ventricular arrhythmias treated by the ICD. In young patients with Brugada syndrome, ICD therapy treated sustained ventricular arrhythmias in 26% of patients over a mean follow-up of 88 months, but 20% experienced inappropriate shocks.