Implantable Cardioverter-Defibrillator Therapy in Brugada Syndrome: A 30-Year Single-Center Experience

Abstract

Background: Brugada syndrome (BrS) continues to pose clinical challenges, despite 3 decades of dedicated research and therapeutic advancements. The pivotal role of implantable cardioverter-defibrillator (ICD) therapy in safeguarding high-risk BrS patients from sudden cardiac death due to ventricular arrhythmias is undeniable. However, the debate on risk stratification and the use of ICDs for primary prevention remains ongoing. Objectives: This study aimed to evaluate the clinical features, management, and long-term outcomes of ICD therapy in patients with Brugada syndrome. Methods: BrS-diagnosed patients were prospectively enrolled. Inclusion criteria were: 1) a Brugada type 1 electrocardiogram pattern, either spontaneous or drug induced; 2) ICD implantation; and 3) consistent follow-up. Risk stratification was based on prior arrhythmic events, and the multiparametric Brussel risk score was used from 2017. High-risk patients underwent video-thoracoscopic epicardial ablation starting in 2016. ICD implantation strategies evolved over time, guided by patients' clinical and demographic characteristics. Results: A total of 306 consecutive Brugada patients (186 male [61%]; mean age 41 ± 17 years; range: 1-82 years) received ICDs at our institution from 1992 to 2022. ICDs were implanted for secondary prevention in 16% of patients. Over the 3 decades, the proportions of secondary prevention implants and asymptomatic patients remained stable, while risk factors fluctuated in the first two decades before stabilizing. During long-term follow-up (median 103 months [63-147 months]), 14% of patients experienced at least 1 sustained ventricular arrhythmia (VA) (1.59 per 100 person-years), 15% had at least 1 inappropriate ICD shock—unaffected by the presence of single or dual leads—and 27% required device revision and/or lead replacement. Patients with secondary prevention ICDs had a higher incidence of both ventricular and supraventricular arrhythmias compared to those with primary prevention ICDs. Loss-of-function mutations and prior nonsustained VAs were associated with sustained VAs. Among high-risk patients, those who underwent epicardial ablation experienced significantly fewer ventricular events. The overall mortality rate was 5.88%, with 22.2% of deaths attributed to cardiac causes. Conclusions: This 30-year study highlights ICD therapy's critical role in preventing fatal arrhythmias in Brugada syndrome, but also reveals frequent device-related complications, especially in younger patients. Thoracoscopic epicardial ablation significantly reduced VA in high-risk patients, offering a promising adjunctive therapy. These findings emphasize the need for individualized treatment strategies to balance the benefits of ICDs with their risks, and underscore the potential of ablation to improve long-term outcomes.