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Brugada Syndrome

DEFINITION

Brugada syndrome is heart rhythm disorder that is abnormal, runs in families, and can be life-threatening. Brugada syndrome is diagnosed or detected with an electrocardiogram. The problem with Brugada syndrome is that it can lead to unstable heart rhythms that can cause fainting and sudden cardiac death. Often, people who have Brugada syndrome don’t have any symptoms and don’t know that they are at risk for cardiac arrest or heart rhythm abnormalities. The heart’s normal pumping motion is coordinated by electrical activity that moves in an organized fashion from the top of the heart (the sinus node) through the middle of the heart (the atrioventricular node) to the bottom of the heart (through the bundle of His and Purkinje fibers). First the electrical impulse reaches the atria at the top which causes them to contract, filling the ventricles with blood. Then, the electrical impulse travels to the bottom of the heart, causing the ventricles to contract, which forces blood out of the heart and through the lungs and body. In Brugada syndrome, the heart has abnormal electrical channels that make this pathway more likely to have a disturbance causing cardiac arrest. When this type of abnormal heartbeat happens, the heart fails to properly pump blood to the body causing lightheadedness or sudden cardiac death. Brugada syndrome most commonly comes from our genetics, but can also be caused by recreational drugs (cocaine), certain prescription medications, structural problems in the heart or abnormalities in electrolytes in the body. Risk factors for Brugada syndrome include having a family member with the syndrome, being a man, and being Asian.

SYMPTOMS
Sudden fainting, sudden cardiac arrest, abnormal heartbeat

DIAGNOSIS
If you have symptoms of fainting or sudden cardiac arrest, your doctor will do an electrocardiogram (EKG or ECG) looking for Brugada Syndrome or other problems with the heart. However, most of the time, Brugada syndrome is found incidentally on electrocardiogram. The electrocardiogram involves placing stickers on the chest attached to a machine by wires that produce a picture of the electrical activity of the heart. From this picture, your doctor can usually tell if you have Brugada syndrome. Sometimes people with Brugada syndrome have a normal electrocardiogram. In that case, the cardiologist may give you a medication through an IV line that helps illicit the abnormal heart rhythm in a controlled setting. If you are found to have Brugada Syndrome, your cardiologist may want you to have testing that can help find the location of the beginning of the abnormal heartbeat, called electrophysiology testing. An electrophysiology (EP) test involves the cardiologist placing a thin catheter with electrodes into a blood vessel in the groin that is fed up to the heart. The electrodes are used to make a map of the electrical activity of the heart and can help your cardiologist determine where the problem is. Finally, your cardiologist may recommend genetic testing for you or your family members which can show if you have Brugada syndrome.

TREATMENT
Treatment options for Brugada Syndrome depend on how serious your Brugada syndrome is. Those at highest risk for a complication from Brugada syndrome are those with a family member who has had sudden cardiac arrest or those who have already had symptoms such as severe fainting episodes and heart rhythm problems. Medications are not effective for the treatment of Brugada syndrome. The only effective treatment is placing an electrical device in the chest called the implantable cardioverter-defibrillator (ICD). This device is placed under the skin in the upper chest with electrical leads going to the heart. Normally, these leads or wires sit in place without doing much. However, if you begin to have an abnormal heart rhythm that leads your heart to not pump blood to the body, the device delivers a shock to restart the heart. In certain circumstances, this shock can be life-saving. Complications of having an ICD placed include bleeding, infection or receiving shocks when you are not having a rhythm abnormality. You should discuss your need for treatment and your treatment options with your doctor or cardiologist.

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