Shanghai Score System
Definition
The Shanghai Score System is a point-based diagnostic framework used to assess the probability of Brugada syndrome (BrS) and early repolarization syndrome (ERS). It was developed to standardize diagnosis by integrating ECG findings, clinical history, family history, and genetic factors.
Key Concepts
- BrS Shanghai Score key criteria: (sources/channelopathies-jaha-2025)
- ECG: Spontaneous type 1 BrS ECG (3.5 pts); fever-induced type 1 (3 pts); type 2/3 converting with drug provocation (2 pts)
- Clinical: Cardiac arrest/VF/PVT (3 pts); nocturnal agonal respirations (2 pts); suspected arrhythmic syncope (2 pts); unexplained syncope (1 pt); AF <30 yrs without alternative cause (0.5 pt)
- Family: Definitive BrS in 1st/2nd-degree relative (2 pts); suspicious BrS-related SCD in relative (1 pt); unexplained SCD in relative <45 yrs (0.5 pt)
- Genetic: Pathogenic BrS mutation (0.5 pt)
- Interpretation: <2 = nondiagnostic; 2–3 = possible BrS; ≥3.5 = probable/definite BrS
- ERS Shanghai Score uses similar structure: ECG J-point elevation ≥0.2 mV with horizontal/descending ST in ≥2 inferior/lateral leads (2 pts); dynamic J-point changes (1.5 pts); ≥0.1 mV J-point in ≥2 leads (1 pt); short-coupled PVCs with R-on-T (2 pts). (sources/channelopathies-jaha-2025)
- Interpretation: <3 = nondiagnostic; 3–4.5 = possible ERS; ≥5 = probable/definite ERS
- At least one ECG finding is required for clinical and family history points to count in both BrS and ERS scoring. (sources/channelopathies-jaha-2025)
- The Shanghai Score was introduced because BrS diagnosis requires more nuanced integration of evidence than the ECG alone, given the variability of spontaneous vs. drug-induced type 1 patterns. (sources/channelopathies-jaha-2025)
- SCB provocation in the context of the Shanghai Score: A positive drug-provoked type 1 ECG scores only 2 points (possible BrS), requiring additional clinical/family history points to reach probable/definite (≥3.5). In practice, a type 1 pattern without an obvious trigger is clearly diagnostic; whether drug-induced type 1 is diagnostic in isolation remains a point of no current consensus — it has direct implications for ICD decisions. (sources/brs-jaccep-2022)
- 2025 EHRA consensus reinforcement: The 2025 EHRA multi-society statement explicitly reaffirms that the Shanghai consensus downgrade of drug-induced type 1 from diagnostic to non-diagnostic is correct practice. A drug-induced type 1 Brugada pattern requires additional relevant symptoms, genetic results, and/or family history to achieve a definite BrS diagnosis. The 2022 ESC VA SCD guidelines state BrS "may be considered" with drug-induced type 1 alone, but confidence increases only when clinical/family features are also present. Isolated drug-induced type 1 (without other features) in an asymptomatic individual with no family history should prompt counselling and lifestyle precautions — but not necessarily ICD implantation. (sources/pharmacological-provocation-europace-2025 — high)
- Polygenic basis of positive SCB response: Positive response to ajmaline or other SCB agents may reflect polygenic susceptibility (common SNP burden), not monogenic BrS — genotype-negative relatives in SCN5A families may have positive tests due to higher polygenic risk scores. This complicates interpretation when a drug-induced type 1 appears in an asymptomatic person with no family history, as it may not represent monogenic BrS requiring intensive management. (sources/pharmacological-provocation-europace-2025 — high)
- High precordial lead positions (V1/V2 at ICS 2–4) increase diagnostic sensitivity ~1.5× compared with standard positions, and ECG testing at both standard and high leads is required for scoring to be valid. (sources/brs-jaccep-2022)
- SCB agents and false-positive risk: Ajmaline is the most potent SCB for provocation; procainamide is least potent. A ~8% false-positive rate for ajmaline was reported in high-risk families (Tadros et al.); ~27% of AVNRT patients and 4.5% of healthy controls showed type 1 ECG changes with ajmaline (Hasdemir et al.) — improved standardization of SCB provocation is needed. (sources/brs-jaccep-2022)
Contradictions / Open Questions
- Low genetic yield for BrS — 0.5 points only: The Shanghai Score assigns only 0.5 points for a pathogenic BrS mutation. In a disease where SCN5A is causal in only 15–30% of cases, genetic positivity carries low diagnostic weight, yet genetic negativity does not lower the score. This asymmetric weighting reflects the diagnostic limitations of genetic testing in BrS and may create false confidence when a pathogenic variant is found. (sources/channelopathies-jaha-2025)
- Spontaneous vs. drug-induced type 1 — key scoring asymmetry: Spontaneous type 1 BrS ECG scores 3.5 points (probable/definite), while drug-induced type 1 scores only 2 points (possible). Drug provocation unmasking is used in a large proportion of BrS diagnoses. The score's implicit assumption that provoked type 1 is less diagnostically certain than spontaneous is not always validated — some provoked patients carry the same arrhythmic risk as spontaneous type 1 carriers. (sources/channelopathies-jaha-2025)
- Ajmaline false-positive rate: Despite ajmaline being the preferred SCB for provocation, a ~8% false-positive challenge rate was reported in BrS families (Tadros et al.), and up to 27% of AVNRT patients showed positive tests (Hasdemir et al.). Standardisation of SCB protocols (dose, stopping criteria) is lacking — the threshold that defines a "positive" test needs further validation. (sources/brs-jaccep-2022)
Connections
- Related to concepts/Schwartz-Score
- Related to entities/Brugada-Syndrome
- Related to entities/Early-Repolarization-Syndrome
- Related to concepts/Pharmacological-Provocation-Testing