Abstract 2824: Clinical Phenotype and Prognosis of Probands With Brugada Syndrome in Relation to SCN5A Mutation. - Japanese Brugada Syndrome Multicenter Registry
Background: A number of studies have reported predictors for prognosis in patients with Brugada syndrome (BrS) including family members, but little is known for the predictors for only probands.
Methods: This multicenter study enrolled 224 probands with BrS (218 males, 47±14 years), in whom SCN5A gene was screened. Inclusion criteria was type 1 ECG at baseline (n=138) or after sodium channel blocker drug challenge test (n=86). The relation between the presence of SCN5A mutation and clinical history, ECG parameters, and cardiac events were investigated.
Results: Compared to patients without mutations (SCN5A(−), n=176), patients with mutations (SCN5A(+), n=48) had the first event at a lower age (34 vs. 43 years, p=0.024), more frequent history of ventricular fibrillation (VF) (33% vs. 18%, p=0.048), higher positive rate of late potential (85% vs. 66%, p=0.015), longer P-wave (128±23 vs. 98±12ms, p<0.001), PQ (223±43 vs. 175±25ms, p<0.001), and QRS (115±21 vs. 94±13ms, p<0.001) durations. During follow up period of 58±32 months, the SCN5A(+) patients and patients with a history of VF experienced more frequent cardiac events (SCN5A; 11/48, 23% vs. 19/176, 11%, P=0.002 (by log rank), history of VF; 22/49, 45% vs. 8/175, 5%, P<0.001 (by log rank)) (Figure⇓). By the multivariate analysis, SCN5A(+) and history of VF were significant predictors for cardiac events (SCN5A(+) vs. SCN5A(−); HR=2.684, p<0.033), history of VF; yes vs. no; HR=4.861, p<0.001). Induced VF at EPS, QRS duration, family history of sudden death, type 1 ECG at baseline were all not a significant predictor.
Conclusion: Japanese probands of BrS with SCN5A gene mutations and history of VF had a higher risk for cardiac events.