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Discussion on Shanghai score for Brugada syndrome.
Shanghai score system for diagnosis of Brugada syndrome was proposed at J Wave Syndromes Consensus Conference held at Shanghai in 2015. Participants included members of HRS, the EHRA and the APHRS.
It was based on available literature and clinical experience of members of the task force.
Scores were based on ECG parameters, clinical history, family history and the results of genetic testing. Highest points in the score was for a spontaneous Type I Brugada ECG which had 3.5 points.
A probable pathogenic mutation in Brugada syndrome susceptibility gene had only 0.5 points. Fever induced type I Brugada pattern and unexplained cardiac arrest or documented ventricular fibrillation/polymorphic ventricular tachycardia had 3 points.
Type 2 or 3 Brugada ECG pattern which converts with provocative drug challenge, nocturnal agonal respirations, suspected arrhythmic syncope and first degree or second degree relative with definite Brugada syndrome had 2 points each. Only the highest score in ECG and clinical history were awarded points.
Syncope of unclear mechanism/etiology and suspicious sudden cardiac death in first or second degree relative had 1 point each. Atrial flutter/fibrillation in patients below the age of 30 years and unexplained sudden cardiac death before the age of 45 years in first/second degree relative with negative autopsy were allotted 0.5 points each.
If the total points were above 3.5, it was considered as probable/definite Brugada syndrome. Between 2-3 points, it was labelled as possible Brugada syndrome. Below 2 points was considered non diagnostic.
An important deviation from the preceding HRS/EHRA/APHRS guidelines was that fever induced or drug induced Brugada pattern requires additional clinical criteria for the diagnosis of Brugada syndrome.
Shanghai score system for diagnosis of Brugada syndrome was validated by Kawada S et al in a study of 393 patients of which 271 were asymptomatic, 99 with syncope and 23 with ventricular fibrillation
They classified patients into 4 groups, with group A having a score 3 points or less, group B with score of 3.5 points, group C with scores between 4 and 5 points and group D with 5.5 points or more.
Group B had 186 patients, groups C and D had 81 patients each, while group A had 45 patients. During a mean follow up period of 97.3 months, 43 patients had ventricular fibrillation.
Statistically significant differences were noted between the groups. Malignant arrhythmic events were not documented in any patient with possible or non-diagnostic Brugada syndrome.
Authors concluded that this study provided validation of Shanghai score system both for the diagnosis and risk stratification of patients with Brugada syndrome.
