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Brugada Syndrome

Definition


Disease: Brugada Syndrome Brugada Syndrome
Category: Cardiovascular diseases
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Disease Definition:

Brugada syndrome, a potentially life-threatening heart rhythm disorder, is usually an inherited condition. This disease is characterized by a specific abnormal heartbeat, which is detected by an electrocardiogram test, called a Brugada sign.

In some people, Brugada syndrome causes dangerous irregular heart rhythms, which can cause sudden cardiac arrest or fainting. However, in others, it doesn’t cause any signs or symptoms, so that they aren't aware of their condition.

Research is still under way on treatments for Brugada syndrome, because this condition was discovered recently. However, Brugada syndrome can be treated by using an implanted medical device called an implantable cardioverter-defibrillator.

Work Group:


Prepared by: Scientific Section

Symptoms, Causes

Symptoms:

Because Brugada syndrome usually doesn’t cause any noticeable signs or symptoms, many people with this condition are not diagnosed.

An abnormal heart rhythm (arrhythmia) called a Brugada sign is the most important sign or symptom of this syndrome. A Brugada sign is a pattern of heartbeats found on a test of the heart rhythm (electrocardiogram or ECG). Because a Brugada sign is only detected on an ECG, a person won't be able to feel it.

The following signs and symptoms indicate that a person has Brugada syndrome:

 

  • Stopped heartbeat, also known as sudden cardiac arrest
  • Irregular heartbeats or palpitations
  • Fainting (syncope)



Seeing a doctor is quite important in order to find out if Brugada syndrome or another heart rhythm problem is causing a person's symptoms, because the signs and symptoms of Brugada syndrome are similar to some other heart rhythm problems.
In some cases, a person may have Brugada sign without having Brugada syndrome.
A person should see a doctor in case they have irregular heartbeat (arrhythmia) or heart palpitations. Tests are necessary in order to make sure that their heart problem is Brugada syndrome, because these palpitations or irregular heartbeats could also be caused by other heart rhythm problems. A person should seek emergency medical attention in case they faint and suspect that it may be due to a heart condition.
A person should also see a doctor in case their sibling, child or parent has been diagnosed with this syndrome. They can discuss with the doctor whether they should undergo genetic testing to see if they're at risk of Brugada syndrome or not.

Causes:

Brugada syndrome is a heart rhythm disorder. Each beat of the heart is triggered by an electrical impulse generated by special cells in the right upper chamber of the heart. Tiny pores, called channels, on each of these cells direct this electrical activity, which makes the heart beat. In Brugada syndrome, a defect in these channels can cause the heart to beat abnormally.
During these episodes, the heart doesn't pump effectively. As a result, not enough blood travels to the rest of the body. This can cause fainting, other heart rhythm disorders, or in extreme cases, sudden cardiac death.

Brugada syndrome is usually inherited, but it may also result from a structural abnormality in the heart, imbalances in chemicals that help transmit electrical signals through the body (electrolytes), or the effects of certain prescription medications or cocaine use.

Brugada syndrome is usually diagnosed in adolescents and adults. It's rarely diagnosed in young children.

Risk factors for Brugada syndrome include:


Family history of Brugada syndrome: If other family members have had Brugada syndrome, one is at an increased risk of having the condition.
Being male: Adult men are more frequently diagnosed than are women. In young children and adolescents, however, boys and girls are diagnosed at about the same rate.
Race: Brugada syndrome occurs more frequently in Asians than in other races.
Fever: While having a fever doesn't bring on Brugada syndrome itself, fever can increase the risk of fainting or other complications of Brugada syndrome, especially in children.

Complications

Complications:

The sudden and unexpected loss of heart function, breathing and consciousness is called a sudden cardiac arrest, a medical emergency and the most serious complication of Brugada syndrome.
This condition can result in sudden cardiac death because if left untreated, it is fatal. However, survival is a possibility with fast and appropriate medical care. In order to improve the chances of survival until emergency personnel arrive, administering CPR (cardiopulmonary resuscitation) or even just rapid compressions to the chest is necessary.

Another complication of Brugada syndrome is sudden fainting (syncope). So, a person should seek immediate medical care in case they have this syndrome and faint.

Treatments:

Treatment of this syndrome depends on the risk of an abnormal heartbeat (arrhythmia). People that are considered at high risk usually have:

 

  • A personal history of serious heart rhythm problems
  • A family history of sudden cardiac death
  • A personal history of severe fainting spells.


Medications cannot treat Brugada syndrome because of the nature of the heart rhythm abnormality. The only thing that can treat this syndrome is a medical device called an implantable cardioverter-defibrillator, which is recommended for people at high risk of sudden cardiac death or some of the other complications of Brugada syndrome.

IMPLANTABLE CARDIOVERTER-DEFIBRILLATOR:
This device will be recommended for people with high-risk. It monitors the heart rhythm continuously and delivers electrical shocks when needed in order to control abnormal heartbeats. To have this device implanted, hospitalization for a day or two is required.
However, ICD implantation has its risks and complications. Some people have reported receiving shocks from their ICD even when their heartbeat was regular. The probable cause of this risk is that the people who receive an ICD as a treatment for Brugada syndrome are generally young, and so they receive shocks during normal stresses, such as exercise, when their heart rate increases. In order to reduce this risk, the ICD should be programmed. The patient should also talk to the doctor about ways to avoid inappropriate shocks in case they have an ICD implanted as part of the treatment of Brugada syndrome.

Prognosis:

Not Available

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