Brugada Syndrome

Question 1

What is Brugada Syndrome?

Answer 1

Brugada Syndrome is a genetic condition characterised by an increased risk of sudden cardiac death due to ventricular arrhythmias.

Question 2

What is the primary cause of Brugada Syndrome?

Answer 2

It is caused by mutations in genes encoding cardiac ion channels, most commonly the SCN5A gene affecting the sodium channel.

Question 3

How is Brugada Syndrome inherited?

Answer 3

It is inherited in an autosomal dominant pattern.

Question 4

What are the characteristic ECG findings in Brugada Syndrome?

Answer 4

Coved ST-segment elevation ≥2 mm in ≥1 of the right precordial leads (V1-V3).

Question 5

What are the three types of Brugada ECG patterns?

Answer 5

Type 1 (coved ST elevation), Type 2 (saddleback ST elevation), and Type 3 (less prominent ST elevation).

Question 6

Which ECG pattern is diagnostic of Brugada Syndrome?

Answer 6

Type 1 coved ST-segment elevation is diagnostic of Brugada Syndrome.

Question 7

What are the common symptoms of Brugada Syndrome?

Answer 7

Symptoms include syncope, palpitations, nocturnal agonal breathing, and sudden cardiac arrest.

Question 8

What is the pathophysiology of Brugada Syndrome?

Answer 8

It involves abnormal sodium channel function, leading to altered repolarisation and predisposition to ventricular arrhythmias.

Question 9

What are the triggers for arrhythmias in Brugada Syndrome?

Answer 9

Fever, certain medications (e.g., sodium channel blockers), excessive alcohol, and electrolyte imbalances.

Question 10

How is Brugada Syndrome diagnosed?

Answer 10

Diagnosis is based on ECG findings, clinical history, and, in some cases, provocative testing with sodium channel blockers.

Question 11

What is the role of a sodium channel blocker test in Brugada Syndrome?

Answer 11

It is used to unmask Type 1 ECG changes in suspected cases where baseline ECG is inconclusive.

Question 12

What are the differential diagnoses for Brugada Syndrome?

Answer 12

Conditions like acute pericarditis, myocardial ischaemia, and electrolyte imbalances can mimic Brugada-like ECG changes.

Question 13

What is the first-line treatment for Brugada Syndrome?

Answer 13

An implantable cardioverter-defibrillator (ICD) is the primary treatment for preventing sudden cardiac death.

Question 14

When is an ICD indicated in Brugada Syndrome?

Answer 14

An ICD is indicated in patients with a history of ventricular arrhythmias, cardiac arrest, or syncope with high-risk features.

Question 15

What lifestyle modifications are recommended for patients with Brugada Syndrome?

Answer 15

Avoidance of fever, certain medications, excessive alcohol, and maintaining normal electrolyte levels.

Question 16

How does fever exacerbate Brugada Syndrome?

Answer 16

Fever increases sodium channel dysfunction, which can unmask Brugada ECG changes and trigger arrhythmias.

Question 17

What medications should be avoided in Brugada Syndrome?

Answer 17

Sodium channel blockers (e.g., flecainide), tricyclic antidepressants, and some anaesthetic agents.

Question 18

What is the role of quinidine in Brugada Syndrome?

Answer 18

Quinidine is used in patients who cannot receive an ICD or as an adjunct therapy to prevent arrhythmias.

Question 19

What are the complications of untreated Brugada Syndrome?

Answer 19

Sudden cardiac death due to ventricular fibrillation or polymorphic ventricular tachycardia.

Question 20

How is syncope in Brugada Syndrome managed?

Answer 20

Syncope is investigated for arrhythmias, and an ICD may be considered if high-risk features are present.

Question 21

What is the prognosis for patients with Brugada Syndrome?

Answer 21

Prognosis varies; patients with an ICD have a significantly reduced risk of sudden cardiac death.

Question 22

Why is genetic testing important in Brugada Syndrome?

Answer 22

Genetic testing helps confirm the diagnosis and facilitates family screening.

Question 23

What is the role of family screening in Brugada Syndrome?

Answer 23

Screening is recommended for first-degree relatives to identify those at risk and offer early management.

Question 24

How is Brugada Syndrome managed during anaesthesia?

Answer 24

Anaesthesia should avoid sodium channel blockers, and close monitoring for arrhythmias is required.

Question 25

Why is early recognition of Brugada Syndrome important?

Answer 25

Early diagnosis and intervention can prevent life-threatening arrhythmias and sudden cardiac death.