Brugada Syndrome Flashcards
What is Brugada Syndrome?
Brugada Syndrome is a genetic condition characterised by an increased risk of sudden cardiac death due to ventricular arrhythmias.
What is the primary cause of Brugada Syndrome?
It is caused by mutations in genes encoding cardiac ion channels, most commonly the SCN5A gene affecting the sodium channel.
How is Brugada Syndrome inherited?
It is inherited in an autosomal dominant pattern.
What are the characteristic ECG findings in Brugada Syndrome?
Coved ST-segment elevation ≥2 mm in ≥1 of the right precordial leads (V1-V3).
What are the three types of Brugada ECG patterns?
Type 1 (coved ST elevation), Type 2 (saddleback ST elevation), and Type 3 (less prominent ST elevation).
Which ECG pattern is diagnostic of Brugada Syndrome?
Type 1 coved ST-segment elevation is diagnostic of Brugada Syndrome.
What are the common symptoms of Brugada Syndrome?
Symptoms include syncope, palpitations, nocturnal agonal breathing, and sudden cardiac arrest.
What is the pathophysiology of Brugada Syndrome?
It involves abnormal sodium channel function, leading to altered repolarisation and predisposition to ventricular arrhythmias.
What are the triggers for arrhythmias in Brugada Syndrome?
Fever, certain medications (e.g., sodium channel blockers), excessive alcohol, and electrolyte imbalances.
How is Brugada Syndrome diagnosed?
Diagnosis is based on ECG findings, clinical history, and, in some cases, provocative testing with sodium channel blockers.
What is the role of a sodium channel blocker test in Brugada Syndrome?
It is used to unmask Type 1 ECG changes in suspected cases where baseline ECG is inconclusive.
What are the differential diagnoses for Brugada Syndrome?
Conditions like acute pericarditis, myocardial ischaemia, and electrolyte imbalances can mimic Brugada-like ECG changes.
What is the first-line treatment for Brugada Syndrome?
An implantable cardioverter-defibrillator (ICD) is the primary treatment for preventing sudden cardiac death.
When is an ICD indicated in Brugada Syndrome?
An ICD is indicated in patients with a history of ventricular arrhythmias, cardiac arrest, or syncope with high-risk features.
What lifestyle modifications are recommended for patients with Brugada Syndrome?
Avoidance of fever, certain medications, excessive alcohol, and maintaining normal electrolyte levels.
How does fever exacerbate Brugada Syndrome?
Fever increases sodium channel dysfunction, which can unmask Brugada ECG changes and trigger arrhythmias.
What medications should be avoided in Brugada Syndrome?
Sodium channel blockers (e.g., flecainide), tricyclic antidepressants, and some anaesthetic agents.
What is the role of quinidine in Brugada Syndrome?
Quinidine is used in patients who cannot receive an ICD or as an adjunct therapy to prevent arrhythmias.
What are the complications of untreated Brugada Syndrome?
Sudden cardiac death due to ventricular fibrillation or polymorphic ventricular tachycardia.
How is syncope in Brugada Syndrome managed?
Syncope is investigated for arrhythmias, and an ICD may be considered if high-risk features are present.
What is the prognosis for patients with Brugada Syndrome?
Prognosis varies; patients with an ICD have a significantly reduced risk of sudden cardiac death.
Why is genetic testing important in Brugada Syndrome?
Genetic testing helps confirm the diagnosis and facilitates family screening.
What is the role of family screening in Brugada Syndrome?
Screening is recommended for first-degree relatives to identify those at risk and offer early management.
How is Brugada Syndrome managed during anaesthesia?
Anaesthesia should avoid sodium channel blockers, and close monitoring for arrhythmias is required.
Why is early recognition of Brugada Syndrome important?
Early diagnosis and intervention can prevent life-threatening arrhythmias and sudden cardiac death.
