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.
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.
Authors: Humberto Rodriguez-Reyes; Andrés Pérez-Riera; Brenda Lucia López; Maria Del Refugio Salinas; Luz Ma Mayela Muñoz; Cesar Ivan Laguna; Nikus Kjell Journal: Ann Noninvasive Electrocardiol Date: 2019-09-30 Impact factor: 1.468
Authors: Estefanía Martínez-Barrios; Sergi Cesar; José Cruzalegui; Clara Hernandez; Elena Arbelo; Victoria Fiol; Josep Brugada; Ramon Brugada; Oscar Campuzano; Georgia Sarquella-Brugada Journal: Biomedicines Date: 2022-01-05
Authors: Maully J Shah; Michael J Silka; Jennifer N Avari Silva; Seshadri Balaji; Cheyenne M Beach; Monica N Benjamin; Charles I Berul; Bryan Cannon; Frank Cecchin; Mitchell I Cohen; Aarti S Dalal; Brynn E Dechert; Anne Foster; Roman Gebauer; M Cecilia Gonzalez Corcia; Prince J Kannankeril; Peter P Karpawich; Jeffery J Kim; Mani Ram Krishna; Peter Kubuš; Martin J LaPage; Douglas Y Mah; Lindsey Malloy-Walton; Aya Miyazaki; Kara S Motonaga; Mary C Niu; Melissa Olen; Thomas Paul; Eric Rosenthal; Elizabeth V Saarel; Massimo Stefano Silvetti; Elizabeth A Stephenson; Reina B Tan; John Triedman; Nicholas H Von Bergen; Philip L Wackel Journal: Indian Pacing Electrophysiol J Date: 2021-07-29