The Brugada Syndrome – a cause of sudden cardiac death!

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This syndrome is responsible for nearly 20% of all sudden cardiac deaths in patients with structurally normal hearts and up to 12% of all sudden cardiac deaths.

                     

What is Brugada syndrome?

It’s a syndrome where abnormal heart rhythms are produced in the heart’s lower chambers (Ventricles). The irregular heartbeat may lead to syncope and sudden cardiac death.

Symptoms of Brugada syndrome

 

Ventricular tachyarrhythmia (a fast, irregular heart rhythm that begins in the lower chambers of the heart) which can lead to fainting palpitations, fluttering in the chest, atrial fibrillation (a fast irregular heart rhythm in the upper chambers of heart), Cardiac arrest and sudden death.

Many people may have this Syndrome but may not show any symptoms. It can be found out on ECG. Brugada syndrome patients often have a recognizable pattern (Brugada pattern) on the ECG.

Causes of Brugada syndrome

It is caused by a genetic mutation which leads to defects in the way the heart’s ion channels work – called channelopathy.

How common is Brugada syndrome?

Brugada syndrome is rare. It affects about 5 of every 10,000 people worldwide. Symptoms often start during adulthood. But the disorder can develop at any age, including infancy. The average age of death related to the disease is 40 years.

Sudden Cardiac Death in Brugada Syndrome

The Brugada syndrome is an inherited channelopathy that alters the main transmembrane ion currents that constitute the cardiac action potential. These changes not only modify the resting electrocardiogram but also predispose patients to develop malignant ventricular tachyarrhythmias that can lead to syncope, cardiac arrest, and sudden cardiac death. This syndrome is responsible for nearly 20% of all sudden cardiac deaths in patients with structurally normal hearts and up to 12% of all sudden cardiac deaths. Brugada syndrome is diagnosed by its characteristic electrocardiogram consisting of a coved type of ST segment elevation of at least 2 mm followed by a negative T wave in either one of the right precordial leads. These changes can be observed spontaneously or after administration of a sodium channel blocker. Primary therapeutic option remains implantation of an implantable cardioverter-defibrillator to avoid sudden cardiac death. Thus, tremendous effort is being made to effectively risk stratify patients to determine who would benefit from implantable cardioverter-defibrillator implantation.

 

Diagnosis of Brugada Syndrome:

A physician’s consultation is a must.

Tests used to diagnose Brugada syndrome include:

1)Electrocardiogram :

Tests interpret electrical activity of the heartbeat. Brugada pattern of waves are produced in the ECG.

2) Electrophysiology testing:

Catheters are placed over the patient’s femoral leg veins which measure the electrical activity from inside the heart. Patients who have an unclear diagnosis require this test.

3)Lab tests like potassium calcium balance imbalance can rule out Brugada Syndrome.

4) Genetic Test for the gene mutation. (The family members can be also tested for this mutation).

Treatment for Brugada Syndrome:

Patients with history of ventricular arrhythmias are at high risk.

A cardiac defibrillator can be implanted in such cases.

Risk factors of Brugada Syndrome:

1)It is commoner in males than females. Men are eight times more likely to develop this condition.

2) Individuals with a family history of Sudden Cardiac Death or Brugada Syndrome, should be screened for the Syndrome.

Family history of Brugada syndrome. This condition is often passed down through families (inherited). Having family members with Brugada syndrome increases a person’s risk of having it.

3)Brugada syndrome occurs more frequently in Asians than in people of other races.

4) Fever doesn’t cause Brugada syndrome, but it can irritate the heart and trigger fainting or sudden cardiac arrest in people with Brugada syndrome, especially children.

Prevention of Brugada Syndrome:

The Physician or a specialist play a vital role in identifying Brugada Syndrome and treating it .

Treatment is by –

1) The Electrophysiologist/Cardiologist who specialises in diagnosing and treating patients with abnormal heart rhythms.

2)Genetic counselors who can study the gene patterns to identify the gene that causes this.

3)A regular follow up with Physician is necessary to monitor medications and health status.

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About the author

Dr.Nikita Pawar is a General Practitioner by profession and has completed her (MBBS PGDCR). She has previously worked in GMC Hospital Dubai & is currently attached to a hospital, corporate company and Clinics in Mumbai. She is passionate about medical article writing and always strives to maintain commitment towards achieving professional growth as she transitions from one phase of her career to the next.

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