Brugada syndrome is an inherited channelopathy that can cause ventricular fibrillation and sudden death in a structurally normal heart. Its type 1 ECG pattern is coved ST elevation of 2 mm or more in V1 or V2 that falls into a negative T wave. Only the type 1 pattern supports the diagnosis.
At a glance
- Type 1, diagnostic: coved ST elevation of 2 mm or more at the J point in V1 or V2, recorded in the 2nd, 3rd or 4th intercostal space, followed by a negative T wave.
- Type 2, suggestive only: saddle-back ST elevation followed by a positive or flat T wave.
- Fever, sodium channel blocking drugs, cocaine and heavy alcohol use can bring out or worsen the pattern.
- When you suspect it, record V1 and V2 one and two intercostal spaces higher.
- Refer anyone with a type 1 pattern to an electrophysiologist. Survivors of cardiac arrest and patients with arrhythmic syncope are candidates for an ICD.
Criteria
Type 1 pattern
Coved ST elevation of 2 mm or more in one or more of V1 and V2, recorded in the 2nd, 3rd or 4th intercostal space, followed by a negative T wave. A spontaneous type 1 pattern is diagnostic; one seen only after a drug challenge needs more evidence, such as arrhythmic syncope or a family history.
Type 2 pattern
Saddle-back ST elevation in V1 or V2 with a positive or flat T wave. Not diagnostic: repeat with high lead positions, during any fever, or with a drug challenge in a specialist center.
Shanghai score
A weighted score of ECG, clinical, family history and genetic findings. 3.5 points or more makes Brugada syndrome probable or definite.
What it looks like
Type 1: coved
Type 2: saddle-back
How to measure it
- Look at V1 and V2 for ST elevation that slopes downward in a coved shape.
- Measure the J point elevation from the baseline. 2 mm or more counts.
- Check the T wave. A negative T wave after a coved ST segment supports type 1.
- If the pattern is suspicious, repeat V1 and V2 one and two intercostal spaces higher.
- Repeat the ECG during any fever: the pattern can appear only then.
Causes
Can bring out or worsen the pattern
- Fever
- sodium channel blockers such as flecainide, ajmaline and procainamide
- tricyclic antidepressants, lithium and some anesthetic drugs
- cocaine and heavy alcohol use
- large meals
- hyperkalemia
Mimics
- Right bundle branch block
- early repolarization and the athlete's heart
- pectus excavatum
- anterior STEMI or right ventricular ischemia
- arrhythmogenic right ventricular cardiomyopathy
- hyperkalemia
- V1 and V2 electrodes placed too high
What to do
- Treat fever promptly with antipyretics in anyone with Brugada syndrome or a suspicious pattern, and repeat the ECG while the fever lasts.
- Advise avoiding drugs that can bring out the pattern; brugadadrugs.org keeps a list.
- Refer a type 1 pattern, or a type 2 pattern with symptoms or a family history of sudden death, to an electrophysiologist.
- Survivors of cardiac arrest or sustained ventricular arrhythmia need an ICD. With a type 1 pattern and syncope that sounds arrhythmic, an ICD is reasonable.
- Screen first-degree relatives with an ECG. Genetic testing finds an SCN5A variant in a minority of patients.
Pitfalls and mimics
- Treating a type 2 pattern as diagnostic.
- Missing a type 1 pattern that shows only in higher intercostal spaces.
- Calling it RBBB: Brugada pattern has no wide S wave in I and V6.
- Ignoring an ECG change during fever: a fever-induced type 1 pattern carries risk.
Key studies
Brugada 1992, J Am Coll Cardiol
Described patients who survived sudden cardiac death with a right bundle branch block-like pattern, persistent ST elevation in V1 to V3 and structurally normal hearts: the first report of the syndrome.
Priori 2013, HRS/EHRA/APHRS expert consensus
Defines Brugada syndrome by a type 1 pattern of 2 mm or more in V1 or V2 in the 2nd to 4th intercostal space, spontaneous or after a sodium channel blocker.
Antzelevitch 2016, J-wave syndromes consensus
Introduced the Shanghai score, which weighs ECG, clinical, family and genetic findings to grade the probability of Brugada syndrome.
Zeppenfeld 2022, ESC ventricular arrhythmia guideline
Current European recommendations on diagnosis, drug avoidance, fever treatment and ICD selection in Brugada syndrome.
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Questions clinicians ask
What does a Brugada ECG look like?
Type 1 shows coved ST elevation of 2 mm or more in V1 or V2 that slopes down into a negative T wave. Type 2 shows saddle-back ST elevation with a positive or flat T wave.
What is the difference between Brugada type 1 and type 2?
Type 1 is coved and diagnostic. Type 2 is saddle-back and only suggestive: it needs high lead positions, a repeat ECG during fever, or a drug challenge in a specialist center.
Can fever cause a Brugada pattern?
Yes. Fever can bring out a type 1 pattern in people with Brugada syndrome and can trigger arrhythmias, so treat fever promptly and repeat the ECG.
Is Brugada syndrome inherited?
Usually it is autosomal dominant with incomplete penetrance. An SCN5A variant is found in a minority of patients, and many have no gene identified, so a negative test doesn't rule it out.
Sources
- Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. J Am Coll Cardiol. 1992;20(6):1391-1396. doi.org/10.1016/0735-1097(92)90253-j
- Priori SG, Wilde AA, Horie M, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):1932-1963. doi.org/10.1016/j.hrthm.2013.05.014
- Antzelevitch C, Yan GX, Ackerman MJ, et al. J-wave syndromes expert consensus conference report: emerging concepts and gaps in knowledge. Heart Rhythm. 2016;13(10):e295-e324. doi.org/10.1016/j.hrthm.2016.05.024
- Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022;43(40):3997-4126. doi.org/10.1093/eurheartj/ehac262
Written in our own words by 2 Minute Medicine's physician editors from the sources listed here. The diagrams are schematic drawings made for 2MM, not patient tracings. This page supports, and does not replace, a full ECG reading and clinical judgment.
For health care professionals. Values are for adults unless a page says otherwise.