Brugada Syndrome

Definition

Definition of Brugada Syndrome
The Brugada syndrome is a genetic disease that is characterised by abnormal electrocardiogram (ECG) findings and an increased risk of sudden cardiac death. It is named by the Spanish cardiologists Pedro Brugada and Josep Brugada. It is the major cause of sudden unexplained death syndrome (SUDS), and is the most common cause of sudden death in young men without known underlying cardiac disease in Thailand and Laos.

Symptoms

Symptoms of Brugada Syndrome
Many people who have Brugada syndrome are undiagnosed because the condition often doesn't cause any noticeable symptoms.

The most important sign or symptom of Brugada syndrome is an abnormal pattern on an electrocardiogram (ECG) called a Brugada sign. A Brugada sign is a pattern of heartbeats that's found on a test of your heart rhythm (electrocardiogram, or ECG). You can't feel a Brugada sign-it's only detected on an ECG.

Causes

Causes of Brugada Syndrome
Brugada syndrome is a heart rhythm disorder. Each beat of your heart is triggered by an electrical impulse generated by special cells in the right upper chamber of your heart. Tiny pores, called channels, on each of these cells direct this electrical activity, which makes your heart beat. In Brugada syndrome, a defect in these channels can cause your heart to beat abnormally.

During these episodes, your heart doesn't pump effectively. As a result, not enough blood travels to the rest of your body. This can cause fainting or sudden cardiac death.

Brugada syndrome is usually inherited, but it may also result from a hard-to-detect structural abnormality in your heart, imbalances in chemicals that help transmit electrical signals through your body (electrolytes), or the effects of certain prescription medications or cocaine use.

Diagnosis

Diagnosis of Brugada Syndrome

• Patients who are symptomatic (unexplained syncopes, ventricular tachycardias or aborted sudden cardiac death) may have a symptom recurrence risk of 2 to 10% per year. In these patients an ICD implant is advisable. Further, life-style advice is given (see below).
• Some groups advise an electrophysiological investigation (inducibility of ventricular fibrillation) for risk assessment in Brugada patients, but others could not reproduce the predictive value of these tests, so the value of inducibility is controversial.
• In large studies familial sudden death did not appear to be a risk factor for sudden death in siblings.
• In asymptomatic patients in whom the Brugada ECG characteristics are present (either spontaneously or provoked by fever or sodium channel blockers like ajmaline, procainimde or flecainide) life style advice is given.

Treatment

Treatment of Brugada Syndrome
The cause of death in Brugada syndrome is ventricular fibrillation. The episodes of syncope (fainting) and sudden death (aborted or not) are caused by fast polymorphic ventricular tachycardias or ventricular fibrillation. These arrhythmias appear with no warning. While there is no exact treatment modality that reliably and totally prevents ventricular fibrillation from occurring in this syndrome, treatment lies in termination of this lethal arrhythmia before it causes death. This is done via implantation of an implantable cardioverter-defibrillator (ICD), which continuously monitors the heart rhythm and will defibrillate an individual if ventricular fibrillation is noted. Some recently performed studies had evaluated the role of quinidine, a Class Ia antiarrhythmic drug, for decreasing VF episodes occurring in this syndrome. Quinidine was found to decrease number of VF episodes and correcting spontaneous ECG changes, possibly via inhibiting Ito channels. Some drugs have been reported to induce the type-1 ECG and/or (fatal) arrhythmias in Brugada syndrome patients. Patients with Brugada syndrome can prevent arrhythmias by avoiding these drugs, or use them only in controlled conditions. Those with risk factors for coronary artery disease may require an angiogram before ICD implantation.

Prognosis

Prognosis of Brugada Syndrome
Consult with your doctor.

Prevention

Prevention of Brugada Syndrome
Consult with your doctor.