Long-term prognosis in patients with non-type 1 Brugada ECG: results from a large Japanese cohort of Brugada syndrome

Background Most recent consensus conference report recommends Brugada syndrome (BrS) is diagnosed in patients with ST segment elevation with spontaneous, drug-induced or fever-induced type 1 morphology. Prognosis in patients with type 2 or 3 ECG without drug-induced or fever-induced type 1 ECG is still unknown. Purpose To evaluate a long-term prognosis in patients with non-type 1 Brugada ECG in a large Japanese cohort of BrS (The Japan Idiopathic Ventricular Fibrillation Study [J-IVFS]). Methods From 528 patients in J-IVFS, a total of 28 consecutive non-type 1 patients (54±14 years, all male, previous sustained ventricular tachyarrhythmias (VTs) 1, syncope 11, asymptomatic 16) were enrolled. Cardiac events (CI: sudden cardiac death or VTs) during the follow-up period were evaluated, and risk factors for the cardiac events were assessed. Results During a mean follow-up period of 111±91 months (median 134 months), 4 patients experienced cardiac events (1.5%/yr), who all had received implantable cardioverter defibrillator implantation. There was no statistically significant clinical risk factor for cardiac events. However, the incidences of cardiac events tended to be higher in symptomatic patients (CI: 25.0, non-CI: 6.3%, p=0.17), those with wide QRS duration >90 msec in lead V2 (CI: 30.0, non-CI: 6.3%, p=0.11), and those with inducible VTs (CI: 21.1, non-CI: 0%, p=0.20), as determined by the Kaplan-Meier method. The annual incidences of cardiac events in patients with symptom, wide QRS duration >90msec in lead V2, or inducible VTs were 2.8, 3.5, and 2.0%/yr, respectively. The incidences of cardiac events were significantly higher in patients with all these 3 factors (9.9%/yr) than those without (p=0.01). Conclusions Our large-scaled multicentre study revealed long-term prognosis in patients with non-type 1 Brugada ECG. The combination of symptom, wide QRS duration in lead V2, and inducible VTs may be useful to evaluate risk for cardiac events. The patients with all these parameters showed high risk for cardiac events and need to be carefully followed. Funding Acknowledgement Type of funding source: None