What is Brugada Syndrome?

Brugada syndrome is an inherited condition called a channelopathy that affects the way electrical signals pass through the heart. It can cause the heat to beat dangerously fast. This is caused by an abnormality in ion channels that affects the electrical state of the heart muscle causing abnormal rhythms that can lead to fainting and sudden death. It affects more men than women.

Effects and Symptoms

Symptoms appear at an average age of 40 years, though they can be present at any age. Fainting and cardiac arrest are the most common symptoms leading to a diagnosis of BrS. Cardiac arrest often occurs during sleep or rest, rather than physical activity. There may be a history of trouble breathing at night. Fever and medications appear to trigger or worsen the symptoms of Brugada Syndrome in many. Some patients, however, show no obvious symptoms. A family history of sudden cardiac death may be present, though not universal, as BrS can occur in an individual with no known family history of the disease.

Diagnosis

BrS can be suspected based on clinical history, family history and physical examination. Characteristic patterns on an ECG can also suggest the diagnosis, or be used to confirm it. These patterns may be present all the time, may appear and then normalize spontaneously, or may be brought on by fever or the administration of particular drugs. BrS may be discovered only when a routine ECG shows an abnormal pattern. 

BrS is sometimes difficult to diagnose. Many people who have a BrS gene may never experience any warning symptoms. The electrical pattern may or may not be seen on an ECG. 

Management and Treatment

There is no cure for BrS, but there are ways to reduce the risk of arrhythmia and sudden death. Patients are advised to immediately treat a fever with anti-fever medications when sick, and to avoid alcohol intake. Many prescription drugs, over-the-counter medications, and drugs used in dental procedures can increase the risk of arrhythmia and should be avoided. A list of drugs to be avoided can be found at this website: www.BrugadaDrugs.org

Those who have symptoms of fainting, seizures or cardiac arrest may be recommended to have an ICD or implantable cardioverter defibrillator surgically placed. An ICD monitors the heart’s activity and can abort an abnormal heart rhythm when it occurs. Though potentially life-saving, an ICD carries risks and complications as well. Careful consideration and discussions are done before an ICD is recommended.