Brugada syndrome is a genetic disorder that causes potentially life-threatening heart rhythm disturbances. Occurring in the lower chambers (ventricles) of your heart, these disturbances can lead to chaotic electrical activity that causes your heart to quiver and fail to pump blood effectively to the rest of your body. The result may be fainting and, eventually, cardiac arrest.
Brugada Syndrome Symptoms and Diagnosis
Fainting (syncope) and cardiac arrest are the most common symptoms of Brugada syndrome; however, some patients do not have any symptoms.
An electrocardiogram (ECG or EKG) can signal Brugada syndrome, but is not always sufficient enough to make a diagnosis. It is possible to have a Brugada pattern on your EKG and not have Brugada syndrome; this pattern can also be caused by a structural abnormality in your heart, abnormal electrolyte levels or side effects of certain medications.
Brugada syndrome is sometimes associated with one or more mutations of the SCN5A gene, but in most cases, the genetic defect is unknown. Risk factors for Brugada syndrome include:
- Family history: A family history of sudden cardiac death.
- Male gender: Men are eight to 10 times more likely to have Brugada syndrome.
- Being Asian or of Asian descent: Brugada syndrome is most common in Asians.
- Age: Brugada syndrome is most commonly seen in men ages 30 to 50.
Brugada Syndrome Treatment at BIDMC
Brugada syndrome has a very poor prognosis if left untreated. The only treatment is an implantable cardioverter defibrillator (ICD), a device that is programmed to detect cardiac arrhythmia and correct it by delivering a jolt of electricity.
Implantation of an ICD is similar to that of a pacemaker: electrode wires are threaded through a vein to the right chambers of the heart, and usually lodged in the apex of the right ventricle.