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What is Brugada Syndrome, sudden cardiac arrest?

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Brugada SyndromeBrugada syndrome is a heart rhythm disorder. Special cells in the right upper chamber of the heart triggers electrical impulses, which are directed by tiny pores known as channels to make a heartbeat. A defect in these channels can cause the heart to beat abnormally and spin electrically out of control in an abnormally fast and dangerous rhythm (ventricular fibrillation) causing Brugada syndrome.

This results in ineffective pumping of the heart that causes insufficiency of blood supply to the rest of the body. This causes fainting if the rhythm lasts for only a short time or sudden cardiac death if the heart remains in that bad rhythm.

Brugada syndrome is a potentially life-threatening heart rhythm disorder that is sometimes inherited. People with Brugada syndrome have an increased risk of abnormal heart rhythms from the lower chambers of the heart (ventricular arrhythmias).

Brugada syndrome is often 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).

How to know if you have Brugada Syndrome?

Most of the people who have Brugada syndrome don’t have any symptoms, and hence they are unaware of this condition. This is more common amongst men. Many people who have Brugada syndrome are undiagnosed because the condition often doesn’t cause any noticeable symptoms. Signs and symptoms of  Brugada syndrome include:

  • Fainting (syncope)
  • Irregular heartbeats or palpitations
  • Extremely fast and chaotic heartbeat (sudden cardiac arrest)

These symptoms are similar to other heart rhythm problems, so it is essential to consult a doctor immediately as it may prove fatal if undiagnosed on time.

The sign more often goes unnoticed and can only be detected on an ECG with abnormal patterns known as type 1 Brugada ECG pattern.

Causes

Brugada syndrome is an unusual genetic disorder of the heart’s electrical system.

Heart’s electrical system requires that the electrical signals be sent through specialized electrical tissue and through the heart muscle itself (called the myocardium). These electrical tissues and the myocardium are able to conduct electricity because of special molecules called ion channels, which allow positively and negatively charged particles to pass through the cells’ walls.

When there is a defect in one of the ion channels, this can lead to a dangerous heart rhythm called ventricular fibrillation, which is a much faster, chaotic heartbeat that sometimes reaches 300 beats a minute. This chaotic heartbeat means very little blood is pumped from the heart to the brain and the body and can be life threatening.  Although people are born with it, they usually do not know they have it until they reach their 30s or 40s.

Who is at more risk?

Brugada syndrome usually is diagnosed in adults and, sometimes, in adolescents. It’s rarely diagnosed in young children.

Risk factors for Brugada syndrome include:

  • Family history of Brugada syndrome –If other family members have had Brugada syndrome, you’re at an increased risk of having the condition.
  • Being male –Adult men are more frequently diagnosed than women. In young children and adolescents, however, boys and girls are diagnosed at about the same rate.
  • Race –Brugada syndrome occurs more frequently in Indians than in other races.
  • Fever –While having a fever doesn’t cause Brugada syndrome by itself, fever can irritate the heart and stimulate a Brugada-triggered faint or sudden cardiac arrest, especially in children.

Complications of Brugada syndrome require emergency medical care. It includes:

  • Sudden cardiac arrest – If not treated immediately, this sudden loss of heart function, breathing and consciousness, which often occurs while sleeping, is fatal. With fast, appropriate medical care, survival is possible.
  • Administering cardiopulmonary resuscitation (CPR) —rapid compressions to the chest — and an external shock from an automatic external defibrillator (AED) can improve the chances of survival until emergency personnel arrive.
  • Fainting (syncope) –If you have Brugada syndrome and you faint, seek emergency medical attention.

When to see a doctor for diagnosis?

Do not ignore any heart palpitations or an irregular heartbeat (arrhythmia), and consult a doctor immediately. The problem could possibly a cause of heart rhythm problem but confirmation through tests can avoid emergency situations. If you faint and you suspect it may be because of a heart condition, seek emergency medical attention.

Apart from typical physical examination, listening to heart with a stethoscope, and the standard 12-lead ECG, other tests include:

  • Electrocardiogram (ECG) with medication – this is a noninvasive test, in which a probe is placed on the chest that records the electrical impulses that make your heart beat. An ECG records these electrical signals and is helpful in detecting irregularities in heart’s rhythm and structure. Medications are given in the form of intravenous injection lines to unmask the abnormal patterns
  • Electrophysiology (EP) test –if the ECG confirm abnormal pattern, EP test is suggested. In an EP test, a catheter is threaded through a vein in the groin to the heart, similar to cardiac catheterization. Electrodes are then passed through the catheter to different points in the heart. The electrodes then map out any irregular heartbeats. The electrodes don’t shock heart — they just detect the electrical signals running through it.

What are the treatments?

Because of the nature of the heart rhythm abnormality, medications usually aren’t used to treat Brugada syndrome. A medical device called an implantable cardioverter-defibrillator is the main treatment.

Implantable cardioverter-defibrillator (ICD) – For high-risk individuals, treatment may include an implantable cardioverter-defibrillator (ICD). This small device continuously monitors the heart rhythm and delivers electrical shocks when needed to control abnormal heartbeats.

To place an ICD, a flexible, insulated wire (lead) is inserted into a major vein under or near the collarbone and guided, with the help of X-ray images, to the heart.

The ends of the leads are secured to the heart’s bottom pumping chambers (ventricles), while the other ends are attached to the shock generator, which is usually implanted under the skin beneath the collarbone. The procedure to implant an ICD requires hospitalisation for a day or two.

(The writer is Dr. Aseem Dhall, Director and Head, Saroj Cardiac Sciences, ISIC, Vasant Kunj. The views expressed are personal opinion.)

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