Shanghai score for Brugada syndrome

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 [1]. Participants included members of Heart Rhythm Society, the European Heart Rhythm Association and the Asian-Pacific Heart Rhythm Society. 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.

If the total points was 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 Heart Rhythm Society/European Heart Rhythm Association/Asia Pacific Heart Rhythm Society guidelines [2] was that fever induced or drug induced Brugada pattern requires additional clinical criteria for the diagnosis of Brugada syndrome [3].

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 [4]. They classified patients into 4 groups, with group A having a score ≤3.0 points, group B with score of 3.5 points, group C with scores between 4 and 5 points and group D with ≥5.5 points. 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 (p = 0.01). 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.

References

1. Antzelevitch C, Yan GX, Ackerman MJ, Borggrefe M, Corrado D, Guo J, Gussak I, Hasdemir C, Horie M, Huikuri H, Ma C, Morita H, Nam GB, Sacher F, Shimizu W, Viskin S, Wilde AA. J-Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge. Heart Rhythm. 2016 Oct;13(10):e295-324.
2. Priori SG, Wilde AA, Horie M, Cho Y, Behr ER, Berul C, Blom N, Brugada J, Chiang CE, Huikuri H, Kannankeril P, Krahn A, Leenhardt A, Moss A, Schwartz PJ, Shimizu W, Tomaselli G, Tracy C. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm. 2013 Dec;10(12):1932-63.
3. Wilde AAM. The Shanghai Score System in Brugada Syndrome: Using it Beyond a Diagnostic Score. JACC Clin Electrophysiol. 2018 Jun;4(6):731-732.
4. Kawada S, Morita H, Antzelevitch C, Morimoto Y, Nakagawa K, Watanabe A, Nishii N, Nakamura K, Ito H. Shanghai Score System for Diagnosis of Brugada Syndrome: Validation of the Score System and System and Reclassification of the Patients. JACC Clin Electrophysiol. 2018 Jun;4(6):724-730.