What are the other Names for this Condition? (Also known as/Synonyms)

• Idiopathic Ventricular Fibrillation, Brugada Type
• Pokkuri Death Syndrome
• Sudden Unexpected Nocturnal Death Syndrome (SUNDS)

What is Brugada Syndrome? (Definition/Background Information)

• Brugada Syndrome is a disorder that affects the lower chambers of the heart leading to irregular heartbeats and disruption of the heart’s normal rhythm. Special channels in the heart are used to conduct electrical activity. A defect in these channels causes the heart defects and other symptoms
• The signs and symptoms of Brugada Syndrome include a special type of electrocardiogram pattern, fainting, and an irregular heartbeat. The condition can lead to sudden unexpected death in some cases
• Research has shown that Asian men are more likely to be affected by this condition, especially later in life. Factors, such as genetic mutations or a genetic predisposition, increase the chances for this condition
• Once Brugada Syndrome is diagnosed, treatment measures, such as an implantable cardioverter-defibrillator (ICD) and drug therapy, may be used. A successful treatment can lead to a good prognosis
• Fainting, cardiac arrest, and ICD-related problems are potential complications that can worsen the prognosis for individuals with Brugada Syndrome
• Brugada Syndrome caused by genetic factors cannot be prevented, but drugs that exacerbate the condition or cause disease-like symptoms may be stopped or discontinued

Who gets Brugada Syndrome? (Age and Sex Distribution)

• Brugada Syndrome usually appears in adulthood, but may shown symptoms at any age
• Males and females of all ethnic and racial backgrounds can be affected
• But, Asian men are more likely to be diagnosed with Brugada Syndrome

What are the Risk Factors for Brugada Syndrome? (Predisposing Factors)

The risk factors for Brugada Syndrome may include:

• Genetic mutations in one of several genes increases the risk of developing the disease
• Genetic predisposition may also increase the chances of an individual developing Brugada Syndrome
• Race: Asian men seem to have a higher risk for some unknown reason

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Brugada Syndrome? (Etiology)

• Brugada Syndrome is caused when special channels in the heart are defective, which leads to improper heartbeats. This can lead to poor blood flow to various parts of the body
• Special cells in the upper right chamber of the heart use channels to create the electrical activity that makes the heart to beat
• Defective channels can be caused by genetic defects, structural abnormalities of the heart, improper electrolyte balance (chemicals that aids in electrical activity are disrupted), etc.
• Certain prescription medicines can also cause Brugada Syndrome-like symptoms or trigger the syndrome
• The condition is inherited in an autosomal dominant fashion, meaning only one version of the mutated gene is needed from either parent. These genes are not well-characterized (i.e., they are still being characterized)

Autosomal dominant: Autosomal dominant conditions are traits or disorders that are present when only one copy of the mutation is inherited on a non-sex chromosome. In these types of conditions, the individual has one normal copy and one mutant copy of the gene. The abnormal gene dominates, masking the effects of the correctly function gene. If an individual has an autosomal dominant condition, the chance of passing the abnormal gene on to their offspring is 50%. Children, who do not inherit the abnormal gene, will not develop the condition or pass it on to their offspring.

What are the Signs and Symptoms of Brugada Syndrome?

The signs and symptoms of Brugada Syndrome may include:

• Arrhythmia or irregular heartbeat
• A unique pattern on an electrocardiogram called a type 1 Brugada pattern
• Fainting
• Fast heartbeats

How is Brugada Syndrome Diagnosed?

The heartbeat irregularities associated with Brugada Syndrome are also similar to irregularities that may occur due to syndromes. Hence, the heartbeat irregularities alone cannot help definitively diagnose Brugada Syndrome.

Brugada Syndrome may be diagnosed by the following tests and exams:

• Thorough analysis of past medical history and physical examination
• Electrocardiogram: A noninvasive test to record and examine the electrical activity of the heart
• Electrophysiology (EP) test: A test to determine or analyze the heart’s electrical activity and establish heart rhythms associated with Brugada Syndrome (if any)
• Genetic testing can be used to detect the mutated genes that are associated with Brugada Syndrome

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Brugada Syndrome?

The possible complications of Brugada Syndrome could include:

• Cardiac arrest: A sudden loss of heart function that usually occurs while sleeping; this can lead to death
• Fall injuries: If individuals faint suddenly, it may result in physical (serious) injuries
• Individuals that have an ICD placed in them may develop infections or occasionally experience inappropriate shocks due to the device

How is Brugada Syndrome Treated?

Brugada Syndrome is treated in the following manner:

• Drug therapy can be used to prevent the heart from going into abnormally dangerous and irregular rhythms
• Implantable cardioverter-defibrillator (ICD): Often used for high-risk individuals, this device helps monitor the heart rhythm and can deliver electrical (controlled) shock pulses, when an abnormal heartbeat is detected. This device helps control the heart rhythm
• Drug therapy can also be used in conjunction with an ICD

Individuals having cardiac arrest due to Brugada Syndrome can be treated by:

• Cardiopulmonary resuscitation (CPR)
• Automatic external defibrillator (AED)

How can Brugada Syndrome be Prevented?

• Currently, Brugada Syndrome caused by genetic factors cannot be prevented, due to it being an inherited disorder
• Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
• If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders such as Brugada Syndrome
• Regular medical screening at periodic intervals with tests, scans and physical examinations are mandatory
• Prescription medicines that cause Brugada-like symptoms or exacerbate Brugada Syndrome should be discontinued

What is the Prognosis of Brugada Syndrome? (Outcomes/Resolutions)

• Appropriate early treatment of Brugada Syndrome can help control the signs and symptoms, which can lead to a good prognosis
• Without treatment, complications may arise, which can lead to a poor prognosis

Additional and Relevant Useful Information for Brugada Syndrome:

The following article links on fainting and heart attacks (first aid) are useful resources for additional information:

https://www.dovemed.com/healthy-living/first-aid/fainting-syncope/

https://www.dovemed.com/healthy-living/first-aid/heart-attack-first-aid/