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UCSF Cardiology
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Department of Medicine

Brugada Syndrome

Brugada syndrome is a genetic condition that causes abnormal heart rhythms (arrhythmias). Individuals with Brugada syndrome can experience syncope (fainting), ventricular tachycardia (fast heart rhythm), ventricular fibrillation (uncoordinated beating of the heart), cardiac arrest, or even sudden death.

The severity of Brugada syndrome varies from person to person even between members of the same family. Some people may never experience symptoms. Others may have serious symptoms, including frequent syncope (fainting) or sudden cardiac death. Most people with Brugada syndrome live a normal life. It can be difficult to predict whether an individual will have a mild form of Brugada syndrome or a more severe form. The risk for cardiac arrest tends to be higher in males, in young adulthood, and when someone has had previous symptoms such as syncope, ventricular tachycardia or cardiac arrest.

Individuals with Brugada often have a characteristic pattern on electrocardiogram (EKG). However, this pattern is only present in about a third of people who have Brugada syndrome, and the abnormal pattern is intermittent. Individuals with Brugada syndrome can have an abnormal EKG at one time and normal EKGs at other times. Sometimes specialized tests are used to diagnose Brugada syndrome. Unfortunately, at this time, there is no test that can always determine who has Brugada syndrome, thus, diagnosing the condition can be difficult.

Treatment for Brugada syndrome can include avoiding certain medications and preventing/aggressively treating fevers and/or implantable cardioverter defibrillator (ICD) depending upon the risk to the individual.

Genetics

The abnormal heart rhythms in Brugada syndrome are due to genetic differences in the electrical system that controls the heart rhythm. Several different genes have been associated with Brugada syndrome but these genes only are responsible for about a third of families with the condition. We expect to identify more genes responsible for Brugada syndrome as our knowledge in cardiac genetics improves over time.

Brugada syndrome is inherited in an autosomal dominant manner. This means that each child of a parent with Brugada syndrome has a 50% chance of inheriting the genetic mutation associated with Brugada. The chance of inheriting it is the same for men and women.

Many people who inherit the genetic mutation never have any health problems related to Brugada syndrome. Family members who carry the genetic mutation associated with Brugada syndrome undergo an evaluation to determine their risk for serious arrhythmias. Treatment may be recommended for those at high risk while those at low risk may only require close follow up and avoidance of certain medications.

Genetic testing can be done to try to identify the genetic mutation causing Brugada syndrome to confirm the diagnosis and so other family members can have genetic testing to determine their risk.

Family members

Since Brugada syndrome is a genetic condition, the family members of someone who has the condition may be at risk of developing the abnormal heart rhythms. Close relatives (parents, siblings, and children) of someone with Brugada syndrome should be evaluated for the condition by consulting a cardiologist familiar with Brugada or through genetic testing if the gene mutation associated with the condition in the family has been found.

Electrophysiologists are cardiologists who are experts in abnormal heart rhythms. Appointments can be made with the electrophysiologists at UCSF by calling (415) 353-2554. Electrophysiologists at other hospitals can be located by searching on this website.

Precautions

Individual recommendations should be made with the help of a cardiologist. Fever and certain medications can trigger the abnormal heart rhythms in Brugada syndrome. Individuals who have Brugada, have an EKG suggestive of Brugada, or carry a genetic mutation for Brugada syndrome are often advised to avoid these medications and fevers. A list of medications to avoid is maintained at www.brugadadrugs.org.

For more information on long QT syndrome: