d o
t o r e g . c o m
Dr. Mete ALPASLAN
Brugada Syndrome
(Sudden Unexpected Nocturnal Death Syndrome)
Etiology
Mutation of SCN5A gene encoding the
sodium channels of cardiac muscle cells.
Less fruquently, mutations involving the
- L-type calcium channel gene
- glycerol-3-phosphate dehydrogenase-like peptide (GPD1-L)
- transient outward potassium current
- ATP-sensitive potassium channel
also have been reported in patients with Brugada syndrome.
Diagnostic criteria
ST segment elevation is observed in right precordial leads C1, C2, C3
(V1, V2, V3).
The ST segment elevation resembles right bundle branch block pattern.
In patients with Brugada Syndrome, the ECG findings may change over time.
Sometimes the ECG may be normal.
Types of Brugada Syndrome according to the ECG appearance
Type 1: 2mm or more coved (concave) ST elevation and negative T wave is seen in at least 2 of the leads V1, V2 or V3. This pattern may be absent at rest and may arise only after administration of a sodium channel blocking agent.
Type 2: Saddleback ST segment elevation is seen. The J point is elevated at least 2mm and the ST segment is elevated at least 1 mm. T wave may be positive (upright) or biphasic.
Type 3: has either a saddleback or coved appearance with an ST-segment elevation of < 1 mm.
A Brugada-like ECG can occasionally appear for a brief period or for a period of several hours after direct-current cardioversion.
For the diagnosis of Brugada Syndrome, at least one of the following should be present
in addition to the typical ECG appearance
Documented ventricular fibrillation (VF).
Documented polymorphic ventricular tachycardia (VT).
A family history of sudden cardiac death before 45 years of age.
Coved-type ECGs in family members.
Inducibility of Documented ventricular tachycardia (VT) with programmed electrical stimulation.
Syncope.
Nocturnal agonal respiration (Video).
Brugada Syndrome is also diagnosed if Type 2 or Type 3 ECG pattern at rest converts to Type 1 after administration of a sodium channel blocking agent. (with the additional requirement of at least one of the above clinical criteria).
Other ECG abnormalities that may be observed in patients with Brugada Syndrome are
Slight prolongation of QT interval.
Prolongation of P wave duration.
Prolongation of PR interval.
Widening of the QRS.
Clinical importance
May result in polymorphic ventricular tachycardia or
ventricular fibrillation.
These arrhythmias may result in syncope or sudden cardiac death.
Ventricular fibrillation in Brugada Syndrome usually occurs during sleep at night.
Although it may be observed at all ages, sudden cardiac death usually occurs at ages of 35-40 years.
About 10% of the patients may show paroxysmal atrial fibrillation.
Brugada syndrome is observed more frequently in males.
In order to decrease the risk of sudden cardiac death, some medicines should be avoided in patients with Brugada Syndrome.
Click here for a list of the drugs that should be avoided in patients with Brugada syndrome.
Factors that modify the appearance of typical ECG findings of Brugada Syndrome
Class 1a, Class 1c and Class 3 antiarhhythmic drugs increase the ST segment elevation.
Adrenergic stimulation decreases ST segment elevation. Vagal stimulation increases ST segment elevation.
In patients with atrial pacing, ST segment elevation increases as heart rate decreases.
Increased body temperature (due to any cause) increases ST segment elevation.
ECG findings similar to Brugada syndrome may also be observed in:
Misplacement of chest electrodes
Myotonic dystrophy
Chagas' disease
Pectus excavatum
Mediastinal tumors
Some drugs including tricyclic antidepressants, fluoxetine, lithium, trifluoperazine, antihistamines, and cocaine.
References (with links to full text and sample ECGs)
Arrhythm Electrophysiol Rev 2018;7(2):135-142.
Arrhythm Electrophysiol Rev 2018;7(2):79-83.
Int J Cardiol Heart Vasc 2018;18:58-64.
Brugada Syndrome.
Arch Cardiovasc Dis 2017;110(3):188-195.
Circ J 2016;80(10):2109-2016.
Pharmacol Ther 2017;176:48-59.
Circulation 2005;111:659-670.
Heart Rhythm 2016;13(7):1497-1503.
Journal of Arrhythmia 2013;29:77-82.
ECG 1. Brugada pattern Type 1.
Dr. Konstantinos Letsas (k.letsas@mail.gr, letsaskpa@yahoo.co.uk) has donated this ECG to our website.
Click here for a more detailed ECG
ECG 2. Another patient with Brugada pattern Type 1.
Dr. Konstantinos Letsas (k.letsas@mail.gr, letsaskpa@yahoo.co.uk) has donated this ECG to our website.
Click here for a more detailed ECG
ECG 3. Brugada pattern Type 2.
Dr. Konstantinos Letsas (k.letsas@mail.gr, letsaskpa@yahoo.co.uk) has donated this ECG to our website.
Click here for a more detailed ECG
ECG 4. Ajmaline Test in Brugada syndrome. The test is considered positive if an additional 1 mm ST segment elevation
appears in leads V1, V2 and V3. The ST segment elevation is measured 0.08 s after the J wave
Dr. Konstantinos Letsas (k.letsas@mail.gr, letsaskpa@yahoo.co.uk) has donated this ECG to our website.
Click here for a more detailed ECG
ECG 5. Type 1 Brugada syndrome.
Click here for a more detailed ECG
ECG 6. Brugada syndrome.
Dr. Ramon Brugada has donated this ECG to our website.
Click here for a more detailed ECG
ECG 7. Brugada syndrome.
Dr. Ramon Brugada has donated this ECG to our website.
Click here for a more detailed ECG
ECG 8. Brugada syndrome.
Dr. Ramon Brugada has donated this ECG to our website.
Click here for a more detailed ECG
ECG 9. Brugada syndrome.
Dr. Ramon Brugada has donated this ECG to our website.
Click here for a more detailed ECG
ECG 10. Brugada syndrome.
Prof. Dr. Haldun Muderrisoglu has donated this ECG to our website.
Click here for a more detailed ECG
Click here to get more information about Brugada Syndrome.
ECG 11. Brugada pattern Type 2. The ECG above belongs to an asymptomatic, apparently healthy 33 years-old male.
Leads V1 and V2 show J point elevation of at least 2 mm and ST segment elevation of at least 1 mm .
Click here for a more detailed ECG
ECG 12a. Brugada pattern Type 3 or not. The ECG above was recorded when the chest electrodes C1 and C2 were placed two
intercostal spaces higher (on the 2nd intercostal space instead of 4th intercostal space).
The above ECG resembles Type 3 Brugada pattern.
Click here for a more detailed ECG
ECG 12b. The ECG above was recorded after correct placement of the chest electrodes C1 and C2 on the 4th intercostal spaces.
Now the Type 3 Brugada pattern is not seen anymore.
Click here for a more detailed ECG
ECG 13. The ECG above belongs to a 31 years-old man who had high fever due to Methicillin-resistant Staphylococcus aureus.
The right precordial leads are derived from 3 ECGs which were recorded at times with different levels of high body temperature.
As body temperature rises, Type 1 Brugada pattern becomes apparent.
The ECG above has been used with the permission of Anatolian Journal of Cardiology and AVES Publishing.
Click here to read the relevant article of Dr. Karabay et al.
(Sudden Unexpected Nocturnal Death Syndrome)
Etiology
Mutation of SCN5A gene encoding the
sodium channels of cardiac muscle cells.
Less fruquently, mutations involving the
- L-type calcium channel gene
- glycerol-3-phosphate dehydrogenase-like peptide (GPD1-L)
- transient outward potassium current
- ATP-sensitive potassium channel
also have been reported in patients with Brugada syndrome.
Diagnostic criteria
ST segment elevation is observed in right precordial leads C1, C2, C3
(V1, V2, V3).
The ST segment elevation resembles right bundle branch block pattern.
In patients with Brugada Syndrome, the ECG findings may change over time.
Sometimes the ECG may be normal.
Types of Brugada Syndrome according to the ECG appearance
Type 1: 2mm or more coved (concave) ST elevation and negative T wave is seen in at least 2 of the leads V1, V2 or V3. This pattern may be absent at rest and may arise only after administration of a sodium channel blocking agent.
Type 2: Saddleback ST segment elevation is seen. The J point is elevated at least 2mm and the ST segment is elevated at least 1 mm. T wave may be positive (upright) or biphasic.
Type 3: has either a saddleback or coved appearance with an ST-segment elevation of < 1 mm.
A Brugada-like ECG can occasionally appear for a brief period or for a period of several hours after direct-current cardioversion.
For the diagnosis of Brugada Syndrome, at least one of the following should be present
in addition to the typical ECG appearance
Documented ventricular fibrillation (VF).
Documented polymorphic ventricular tachycardia (VT).
A family history of sudden cardiac death before 45 years of age.
Coved-type ECGs in family members.
Inducibility of Documented ventricular tachycardia (VT) with programmed electrical stimulation.
Syncope.
Nocturnal agonal respiration (Video).
Brugada Syndrome is also diagnosed if Type 2 or Type 3 ECG pattern at rest converts to Type 1 after administration of a sodium channel blocking agent. (with the additional requirement of at least one of the above clinical criteria).
Other ECG abnormalities that may be observed in patients with Brugada Syndrome are
Slight prolongation of QT interval.
Prolongation of P wave duration.
Prolongation of PR interval.
Widening of the QRS.
Clinical importance
May result in polymorphic ventricular tachycardia or
ventricular fibrillation.
These arrhythmias may result in syncope or sudden cardiac death.
Ventricular fibrillation in Brugada Syndrome usually occurs during sleep at night.
Although it may be observed at all ages, sudden cardiac death usually occurs at ages of 35-40 years.
About 10% of the patients may show paroxysmal atrial fibrillation.
Brugada syndrome is observed more frequently in males.
In order to decrease the risk of sudden cardiac death, some medicines should be avoided in patients with Brugada Syndrome.
Click here for a list of the drugs that should be avoided in patients with Brugada syndrome.
Factors that modify the appearance of typical ECG findings of Brugada Syndrome
Class 1a, Class 1c and Class 3 antiarhhythmic drugs increase the ST segment elevation.
Adrenergic stimulation decreases ST segment elevation. Vagal stimulation increases ST segment elevation.
In patients with atrial pacing, ST segment elevation increases as heart rate decreases.
Increased body temperature (due to any cause) increases ST segment elevation.
ECG findings similar to Brugada syndrome may also be observed in:
Misplacement of chest electrodes
Myotonic dystrophy
Chagas' disease
Pectus excavatum
Mediastinal tumors
Some drugs including tricyclic antidepressants, fluoxetine, lithium, trifluoperazine, antihistamines, and cocaine.
References (with links to full text and sample ECGs)
Arrhythm Electrophysiol Rev 2018;7(2):135-142.
Arrhythm Electrophysiol Rev 2018;7(2):79-83.
Int J Cardiol Heart Vasc 2018;18:58-64.
Brugada Syndrome.
Arch Cardiovasc Dis 2017;110(3):188-195.
Circ J 2016;80(10):2109-2016.
Pharmacol Ther 2017;176:48-59.
Circulation 2005;111:659-670.
Heart Rhythm 2016;13(7):1497-1503.
Journal of Arrhythmia 2013;29:77-82.
ECG 1. Brugada pattern Type 1.
Dr. Konstantinos Letsas (k.letsas@mail.gr, letsaskpa@yahoo.co.uk) has donated this ECG to our website.
Click here for a more detailed ECG
ECG 2. Another patient with Brugada pattern Type 1.
Dr. Konstantinos Letsas (k.letsas@mail.gr, letsaskpa@yahoo.co.uk) has donated this ECG to our website.
Click here for a more detailed ECG
ECG 3. Brugada pattern Type 2.
Dr. Konstantinos Letsas (k.letsas@mail.gr, letsaskpa@yahoo.co.uk) has donated this ECG to our website.
Click here for a more detailed ECG
ECG 4. Ajmaline Test in Brugada syndrome. The test is considered positive if an additional 1 mm ST segment elevation
appears in leads V1, V2 and V3. The ST segment elevation is measured 0.08 s after the J wave
Dr. Konstantinos Letsas (k.letsas@mail.gr, letsaskpa@yahoo.co.uk) has donated this ECG to our website.
Click here for a more detailed ECG
ECG 5. Type 1 Brugada syndrome.
Click here for a more detailed ECG
ECG 6. Brugada syndrome.
Dr. Ramon Brugada has donated this ECG to our website.
Click here for a more detailed ECG
ECG 7. Brugada syndrome.
Dr. Ramon Brugada has donated this ECG to our website.
Click here for a more detailed ECG
ECG 8. Brugada syndrome.
Dr. Ramon Brugada has donated this ECG to our website.
Click here for a more detailed ECG
ECG 9. Brugada syndrome.
Dr. Ramon Brugada has donated this ECG to our website.
Click here for a more detailed ECG
ECG 10. Brugada syndrome.
Prof. Dr. Haldun Muderrisoglu has donated this ECG to our website.
Click here for a more detailed ECG
Click here to get more information about Brugada Syndrome.
ECG 11. Brugada pattern Type 2. The ECG above belongs to an asymptomatic, apparently healthy 33 years-old male.
Leads V1 and V2 show J point elevation of at least 2 mm and ST segment elevation of at least 1 mm .
Click here for a more detailed ECG
ECG 12a. Brugada pattern Type 3 or not. The ECG above was recorded when the chest electrodes C1 and C2 were placed two
intercostal spaces higher (on the 2nd intercostal space instead of 4th intercostal space).
The above ECG resembles Type 3 Brugada pattern.
Click here for a more detailed ECG
ECG 12b. The ECG above was recorded after correct placement of the chest electrodes C1 and C2 on the 4th intercostal spaces.
Now the Type 3 Brugada pattern is not seen anymore.
Click here for a more detailed ECG
ECG 13. The ECG above belongs to a 31 years-old man who had high fever due to Methicillin-resistant Staphylococcus aureus.
The right precordial leads are derived from 3 ECGs which were recorded at times with different levels of high body temperature.
As body temperature rises, Type 1 Brugada pattern becomes apparent.
The ECG above has been used with the permission of Anatolian Journal of Cardiology and AVES Publishing.
Click here to read the relevant article of Dr. Karabay et al.