How Is the Shanghai Score for Brugada Syndrome Calculated?

If you are looking at your cardiology notes and see a reference to the “Shanghai Score,” you might wonder what this number means. The Shanghai Scoring System is a standardized checklist or point system that cardiologists use to diagnose Brugada syndrome ^[1]. Because diagnosing Brugada syndrome can be complex, this system brings together different pieces of the puzzle—including your electrocardiogram (ECG) results, personal medical history, family history, and genetic test results—to calculate the probability that you have the condition ^[1][2].

To use the Shanghai Score, a doctor tallies up points based on your specific findings ^[1]. However, there is a very important rule: doctors only count the single highest-value finding within each category ^[1]. For example, if you have two different findings in the “Personal Clinical History” category, only the finding with the higher score is added to your total ^[1].

A total score of 3.5 points or higher means you have a “probable” or “definite” diagnosis of Brugada syndrome ^[1][3]. A score of 2 to 3 points means “possible” Brugada syndrome, and a score of less than 2 is considered non-diagnostic ^[1].

How the Points are Tallied

The system awards points across four main categories. The more strongly a specific finding points to Brugada syndrome, the more points it receives. Remember, only your highest score in each category counts toward your final total.

1. Electrocardiogram (ECG) Findings

Your ECG, which records the electrical signals in your heart, is the most important part of the diagnosis.

• Spontaneous Type 1 Brugada pattern: If your ECG naturally shows the classic “Type 1” Brugada pattern without any medications or triggers, you are awarded 3.5 points ^[1]. This alone is enough to confirm the diagnosis ^[1][4].
• Fever-induced Type 1 Brugada pattern: If the Type 1 pattern appears only when you have a high fever, you receive 3.0 points ^[1]. This makes fever an important trigger that Brugada patients must carefully monitor and treat.
• Drug-induced Type 1 Brugada pattern: If your baseline ECG is normal, but the Type 1 pattern appears during a provocative test (a test where you are given a medication like ajmaline or flecainide to see how your heart reacts), you are awarded 2.0 points ^[5][6].

2. Personal Clinical History

Doctors also look at your personal symptoms and medical events.

• Cardiac Arrest or Serious Rhythms: Having a documented history of unexplained cardiac arrest or dangerous fast heart rhythms (like ventricular fibrillation, where the lower chambers of the heart quiver instead of pumping blood) gives 2.0 points ^[1][2].
• Unusual Breathing at Night: Episodes of noisy, gasping breathing while you sleep, known as nocturnal agonal respiration, are worth 1.2 points ^[1][2].
• Arrhythmic Syncope: A history of syncope (fainting) suspected to be caused by an irregular heartbeat is worth 1.0 point ^[1].

3. Family History

Because Brugada syndrome can be inherited, your family’s medical history is a key clue.

• Definite Brugada Syndrome in the Family: If a first-degree relative (parent, sibling, or child) has a definite Brugada syndrome diagnosis, you get 2.0 points ^[1][2].
• Sudden Cardiac Death: If a first-degree relative passed away from sudden cardiac death before the age of 45, you receive 1.0 point ^[1][2].

4. Genetic Test Results

While genetic testing is helpful, it is just one part of the puzzle.

• SCN5A Mutation: Testing positive for a known disease-causing mutation in the SCN5A gene (the most common genetic link to Brugada syndrome) adds 0.5 points ^[1][7].

It is important to know that genetic testing is only positive in about 20% to 30% of patients with Brugada syndrome ^[8][7]. Because the mutation is only found in a minority of patients, it only contributes a small amount to the total score ^[2]. A negative genetic test does not mean you do not have Brugada syndrome.

What Your Score Means for Your Care

Adding up these points helps your care team confidently decide on a diagnosis. If your score is 3.5 or higher, the diagnosis is clear ^[1]. Once confirmed, your doctor will discuss actionable next steps to protect your heart, such as creating a list of medications you must avoid, aggressively managing fevers with medication, or discussing whether you need an implantable cardioverter-defibrillator (ICD) (a device placed in your chest to shock your heart back into a normal rhythm if a dangerous rhythm occurs) ^[1][9].

If your score falls in the “possible” range (2 to 3 points), your doctor may suggest further testing, closer monitoring, or evaluation of other family members to gather more information ^[1][9]. While the Shanghai Score is an excellent diagnostic tool, cardiologists continue to use it alongside their clinical judgment to ensure you receive the safest and most personalized care ^[10][9].