Drug-induced Brugada Syndrome in Children: Clinical Features, Device-based Management and Long-Term Follow-up.

OBJECTIVES:

To investigate clinical features, management and long-term follow-up of children with drug-induced Brugada syndrome (BS).

BACKGROUND:

BS patients younger than 12 years with spontaneous type I ECG have a higher risk of arrhythmic events. Data on drug-induced BS in patients younger than 12 years are lacking.

METHODS:

Among 505 patients with ajmaline-induced BS, individuals 12 years of age or younger at the time of diagnosis were considered as children and eligible for this study.

RESULTS:

Forty children (60% male, mean age 8±2.8 years) were included. A family history of sudden death (SD) was present in 24 children (60%). Two (5%) had a previous episode of aborted SD and 8 (20%) had syncope. Children experienced more frequent episodes of sinus node dysfunction (SND) as compared to older individuals (7.5% vs 1.5%, p=0.04) and had a comparable incidence of atrial tachyarrhythmias. Children experienced more frequently episodes of ajmaline-induced sustained ventricular arrhythmias (VAs) as compared to older patients (10% vs 1.3%, p=0.005). Twelve children (30%) received an implantable cardioverter defibrillator (ICD). After a mean follow-up time of 83±51 months, none of the children died suddenly. Spontaneous sustained VAs were documented in 1 child (2%). Among children with ICD, 1 (8%) experienced an appropriate shock, 4 (33%) had inappropriate ICD shocks and 4 (33%) experienced device-related complications.

CONCLUSIONS:

Drug-induced BS in children is associated with atrial arrhythmias and SND. Children are at higher risk of ajmaline-induced VAs. The rate of device-related complications, leading to lead replacement or inappropriate shocks, is considerable and even higher than appropriate interventions. Based on these findings, the optimal management of BS in childhood should remain individualized taking into consideration patient's clinical history and family's wishes.