Late life‐threatening arrhythmia in patients with Brugada syndrome: Results from long‐term follow‐up in a large Japanese cohort

• Published in: Journal of cardiovascular electrophysiology Vol. 35; no. 4; pp. 701 - 707
• Main Authors: Shinohara, Tetsuji, Takagi, Masahiko, Kamakura, Tsukasa, Komatsu, Yuki, Aizawa, Yoshiyasu, Sekiguchi, Yukio, Yokoyama, Yasuhiro, Aihara, Naohiko, Hiraoka, Masayasu, Aonuma, Kazutaka
• Format: Journal Article
• Language: English
• Published: United States Wiley Subscription Services, Inc 01.04.2024
• Subjects: Arrhythmia; Asymptomatic; Brugada syndrome; Cardiac arrhythmia; Electrical stimuli; Heart; late life‐threatening arrhythmia; programmed electrical stimulation; Risk factors; risk stratification; Tachyarrhythmia; Tachycardia; Ventricle; ventricular fibrillation; programmed electrical stimulation; ventricular fibrillation; risk stratification; Brugada syndrome; late life‐threatening arrhythmia
• ISSN: 1045-3873; 1540-8167
• DOI: 10.1111/jce.16205

Introduction Most patients with Brugada syndrome (BrS) are first diagnosed in their 40s, with sudden cardiac death (SCD) often occurring in their 50s. Ventricular fibrillation (VF) may occur in some patients with BrS despite having been asymptomatic for a long period. This study aimed to assess the incidence and risk factors for late life‐threatening arrhythmias in patients with BrS. Methods Patients with BrS (n = 523; mean age, 51 ± 13 years; male, n = 497) were enrolled. The risk of late life‐threatening arrhythmia was investigated in 225 patients who had experienced no cardiac events (CEs: SCD or ventricular tachyarrhythmia) for at least 10 years after study enrollment. The incidence of CEs during the follow‐up period was examined. Results During the follow‐up of the 523 patients, 59 (11%) experienced CEs. The annual incidences of CEs were 2.87%, 0.77%, and 0.09% from study enrollment to 3, 3−10, and after 10 years, respectively. Among 225 patients who had experienced no CEs for at least 10 years after enrollment, four patients (1.8%) subsequently experienced CEs. Kaplan–Meier analysis revealed significant differences in the incidence of late CEs between patients with and without a history of symptoms (p = .032). The positive and negative predictive values of late CEs for the programmed electrical stimulation (PES) test were 2.9% and 100%, respectively. Conclusion Our results suggest that patients with BrS who are asymptomatic and have no ventricular tachycardia/VF inducibility by PES are at extremely low risk of experiencing late life‐threatening arrhythmias. Patients with Brugada syndrome who are asymptomatic and have no ventricular tachycardia/ventricular fibrillation inducibility by programmed electrical stimulation are at extremely low risk of experiencing late life‐threatening arrhythmias.