Brugada syndrome (BrS) is a channelopathy that can also cause dangerous ventricular arrhythmias. Patients with BrS can have an abnormal pattern on their ECG. Brugada syndrome is the rarest of the channelopathies and usually presents when a person is in their 30's to 40's. However, it is often identified when family members of patient with BrS are being tested, or when someone is having an ECG for a non-cardiac reason, such as a sports physical. There is no treatment that completely prevents episodes of ventricular arrhythmias, but medications affecting sodium channels in the heart may be used.
Symptoms and Signs
Symptoms of Brugada Syndrome (BrS) may include dizziness, fainting, sudden death, or no symptoms at all. Development of a fever appears to trigger BrS events, so aggressive treatment of fever in addition to close monitoring, is warranted for all BrS patients regardless of their symptoms or the presence of an implantable cardioverter defibrillator (ICD).
In addition to the ECG findings, an IV medication can be given while the patient is being monitored to help aid with the diagnosis. This procedure is called a medication challenge.
As with LQTS, patients with BrS should avoid medications known or suspected to provoke BrS events whenever possible. A list of drugs to avoid can be found at www.brugadadrugs.org.