Brugada Syndrome (BrS) in Children
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).
An ECG may be helpful in the diagnosis of Brugada syndrome. In some instances, the ECG can show "Brugada pattern" which can be diagnostic. A normal ECG does not rule out Brugada syndrome.
A drug (medication) infusion study can help the electrophysiology team determine if patients have certain kinds of cardiac (heart) genetic syndromes or channelopathies.
Genetic testing and counseling can be valuable tools when evaluating patients and their family members for channelopathies.
Treatment of Brugada syndrome focuses on preventing and treating ventricular arrhythmias. Implantation of an implantable cardioverter defibrillator (ICD or defibrillator) may be indicated for certain patients, especially those who have had a life-threatening arrhythmia (such as ventricular tachycardia or ventricular fibrillation).
In patients with Brugada syndrome, a fever can increase the risk of ventricular arrhythmia. It is therefore important to promptly treat fever with medications that reduce fever such as ibuprofen and acetaminophen. Your doctor will discuss this recommendation with you.
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 BrugadaDrugs.org.
For more information about living with this condition, visit SADS.org.