Shanghai Score System for Diagnosis of Brugada Syndrome: Validation of the Score System and System and Reclassification of the Patients

Objectives: The principal objective was to perform an initial test of the Shanghai Brugada Scoring System. Diagnosis of probable and/or definite Brugada syndrome (BrS), possible BrS, and nondiagnostic outcomes were assigned scores of ≥3.5, 2 to 3, and <2 points, respectively. The proposed score system was based on the available published reports and on weighted coefficients derived from limited datasets, with the understanding that these recommendations would need to undergo continuing validation. Background: The 2016 HRS/EHRA/APHRS/SOLAECE J-Wave Syndrome Consensus Report proposed a scoring system for diagnosis of BrS that takes into account electrocardiographic recordings, genetic results, clinical characteristics, and family history. Methods: The patient population consisted of 393 patients evaluated at our hospital for BrS (271 asymptomatic, 99 with syncope, and 23 with ventricular fibrillation [VF]) between 1996 and 2016. Subjects were classified into 4 groups: group A with a score of ≤3.0 points (n = 45); group B with a score of 3.5 points (n = 186); group C with a score of 4.0 to 5.0 points (n = 81); and group D with a score of ≥5.5 points (n = 81). Results: A total of 348 (88%) patients had probable and/or definite BrS, and 81 (20%) had a score ≥5.5. During a follow-up of 97.3 months (range: 39.7 to 142.1 months), 43 patients experienced VF. Significant differences were seen among the 4 groups (p = 0.01). A malignant arrhythmic event did not occur in any patient with possible or nondiagnostic BrS. Conclusions: This study provided validation for the use of the Shanghai Score System for the diagnosis and risk stratification of patients with BrS.