2 min read - Brugada Syndrome

An 18 years old schoolboy came with epigastric burning pain for 2 days duration. He had similar episodes in the past which responded to over the counter antacids.

This ECG was taken.



What is it?

Brugada syndrome. Well the nomenclature is confusing. But as long as you are not a cardiologist, call it Brugada Syndrome!



What is Brugada syndrome?

It is an autosomal dominant genetic disorder. Mutation is in a cardiac sodium channel. It leads to an abnormal ECG pattern called 'Brugada pattern' which carries an increased risk of ventricular arrhythmias and sudden cardiac deaths.


Is it common? Do I really have to know it?

It's not uncommon. Prevalence can be up to 1%, but could be even more as there can be many undetected people.

But, if you miss it the consequences can be catastrophic!



What are the ECG features?

There are two patterns.


Type 1 pattern is diagnostic.

There will be 'coved' ST elevation of more than 2mm in one or more of lead V1-V3.


Type 2 pattern is suggestive.



Are these ECG changes always present?

No. Sometimes this pattern is seen only when provoked. Provoking factors include fever and many drugs. Even coccaine can unmask it!



What are the clinical manifestations?

Many remain asymptomatic. Patients can have palpitations and syncopal episodes. Some can have transient arrhythmias in the night leading to abnormal gasping like respiratory pattern (Nocturnal agonal breathing). For some, unfortunate first event is the sudden cardiac death itself.



What should you do when you see this in a patient?

It's always better to exclude acute MI if you are not certain.

You basically can't manage this patient! Refer the patient early to a cardiologist or an electrocardiophysiologist.


What will they do?

There are many protocols. But ultimately they will decide either to start a drug, put the patient on an ICD (Implantable Cardioverter-Defibrillator - an implantable gadget that will shock the patient whenever they get an arrhythmia) or leave the patient alone.

Should you give any advice until the patient sees a cardiologist?

It's safe to avoid high intensity competitive sports. 


Anything else?

Screening of first degree relatives is essential.



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