Brugada Syndrome

 

Shanthi Ravichandran

DNO (Deputy Nursing Officer), Sri Ramakrishna Hospital, Coimbatore.

*Corresponding Author E-mail:  Shanthi.dno@sriramkrishnahosiptal.co.in

 

ABSTRACT:

Brugada syndrome (BrS) is an inherited ion channel channelopathy predisposing to ventricular arrhythmias and sudden cardiac death. Originally believed to be predominantly associated with mutations in SCN5A encoding for the cardiac sodium channel, mutations of 18 genes other than SCN5A have been implicated in the pathogenesis of BrS to date. Diagnosis is based on the presence of a spontaneous or drug-induced coved-type ST segment elevation. If untreated, the irregular heartbeats even can sudden death.

 

KEYWORDS:  Brugada syndrome, BrS, Icd, Arrhythimias Ion channel, Repolarization, Depolarization, Sudden death, Ion channels, Conduction (block).

 

 


INTRODUCTION:

Brugada syndrome (BrS) is a rare heart condition that can make your heart’s lower chambers (ventricles) beat in an abnormal way. This can make you faint or have a cardiac arrest. Treatments include medicine or a medical device that delivers a shock when you need it.

 

DEFINITION:

Brugada syndrome is a rare condition that causes an abnormal heart rhythm in your heart's lower chambers (ventricles). This irregular heartbeat can cause fainting (syncope) and lead to sudden cardiac death (SCD). This often happens while you’re at rest or asleep. Cardiologists identified Brugada syndrome in 1992. Researchers continue to study the condition and test new treatments.

 

INCIDENCE:

Brugada syndrome is more common in people assigned male at birth. Men are eight to 10 times more likely than women to have the condition. Anyone with a family history of SCD or Brugada syndrome should find out if they have the disease. Symptoms often start when you’re in your 30s or 40s.An estimated 5 people out of 10,000 have the condition.

 

CASE HISTORY:

Mr.Kasiviswanathan 49 years old male was admitted on 28.06.22 at 8.30pm at Sri Ramakrishna Hospital with complaints of self fall followed by giddiness .Initially patient was treated as  ACS/NSTEMI  at outside hospital and administered loading dose of Tab. Deplatt 600mg Tab. Brillianta 180mg,Tab.Ecosprin 325 mg, Tab. Flavedon MR 35 mg, Tab. Atrova 80 MG & Inj. Heparin 5000 units as stat dose and for further treatment the patient was referred to SRH for further Management. with the help of ecg patient diagnosed as a burgada syndrome and treated with implantable cardiac defebrilator.

 

Symptoms and Causes:

Brugada syndrome symptoms can happen at any age and may include:

·      Ventricular arrhythmia

·      Syncope.

·      Dizziness.

·      Difficulty breathing.

·      Seizures.

·      Heart palpitations.

·      Atrial fibrillation

·      Cardiac arrest.

 

Other symptom triggers include:

·      Heat exhaustion.

·      Dehydration.

·      Sodium channel blockers

·      Lithium.

·      Tricyclic antidepressants.

·      Drinking too much alcohol.

·      Cocaine.

·      Marijuana.

 

About 70% of people with Brugada syndrome don’t have a known genetic mutation. However, others have a genetic mutation in one of 18 or more genes. These mutations interfere with heartbeat signal conduction in your heart.

 

People without a genetic mutation may get Brugada syndrome from an unknown cause or from medicines they take for certain mental health conditions or heart issues. It only takes one copy of an affected gene from one parent to inherit Brugada syndrome. Any child of someone with a Brugada-related gene mutation has a 50% chance of having it too.

 

To make a Brugada syndrome diagnosis, a healthcare provider will:

·      Perform a physical exam.

·      Review your medical history.

·      Review your family history, especially a history of sudden cardiac death.

·      Order tests.

 

Tests for diagnosing Brugada syndrome include:

·      Genetic testing:

This can confirm that you have a specific genetic mutation that indicates Brugada syndrome. Other family members may want to have a test for this mutation.

·      Electrocardiogram (EKG):

This test records the electrical activity that makes your heart beat. Because a pattern typical of Brugada syndrome may not appear at first, a provider may immediately do the test two more times. They reposition the leads between tests to try to capture the pattern. They also may do an EKG before and after you eat a large meal.

·      EKG with medication:

Before doing an EKG, your provider may give you medication that helps the Brugada pattern show up on the EKG printout if you have the condition. Your provider may call this a drug challenge test.


 

      


Based on your EKG results, you may also have:

·      Electrophysiology (EP) testing:

Providers place catheters inside your heart through your femoral (leg) veins. These catheters measure electrical activity from inside your heart. You may have this test if your diagnosis is unclear after earlier tests.

·      Lab tests: These check for a normal potassium-calcium balance and can rule out other causes of an abnormal heart rhythm.

 

Management:

·      Isoproterenol.

·      Quinidine.

·      Amiodarone.

·      Implantable cardioverter defibrillator (ICD) if you’re at a high risk for ventricular fibrillation, have fainting spells or survived a cardiac arrest.

·      Ablation (in some cases).

 

Special care and life style for burgada syndrome

The first line of treatment, suitable for all people with Brugada syndrome regardless of their risk of arrhythmias, is lifestyle advice. People should be advised to recognise and avoid things that may increase the risk of serious arrhythmias. Brugada syndrome is a serious condition that some people die from. But the chances of this happening can be significantly reduced if it's diagnosed and treated. You'll need to avoid things that can trigger the condition and have regular check-ups. Most people are able to carry out everyday activities, such as:

·      Exercise

·      Driving

·      Having Sex

·      Getting pregnant and having children

 

CONCLUSION:

The Brs  is an inherited primary arrhythmia syndro meoriginally thought to involve structurally normal hearts. Recent evidence implicates structural alterations of fibrotic change in the right ventricle. Risk stratification is based on a combination of genetic studies, symptoms, the presence of spontaneous or induced Brugada pattern on the ECG, ECG conduction and repolarization parameters as well as programmed electrical stimulation procedures to test for VT inducibility. High risk patients require ICD implantation. New developments such as subcutaneous ICDs might reduce the complication rates of transvenous ICDs, but its use is limited by the considerable rate of sensing screening failure. If electrophysiological substrates arising from the RVOT are confirmed by mapping, they can be eliminated using catheter ablation.

 

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Received on 02.03.2023        Modified on 18.05.2023

Accepted on 20.07.2023       ©A&V Publications All right reserved

A and V Pub IntJ. of Nursing and Medical Res. 2023; 2(3):100-102.

DOI: 10.52711/ijnmr.2023.24