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Brugada Syndrome:
Brugada syndrome is a rare but potentially life-threatening inherited disease that predisposes patients to fatal cardiac arrhythmias.
The syndrome is characterized by the ECG findings of a right bundle branch block and ST-segment elevations in the right precordial leads (V1 to V3).
Genetically, Brugada syndrome is primarily linked to mutations in the SCN5A gene, accounting for 15 to 30% of cases.
However, its inheritance follows an autosomal dominant pattern with variable expressivity and reduced penetrance.
Environmental factors, such as temperature, certain medications, electrolyte imbalances, and cocaine use, can influence its presentation.
The prevalence of Brugada Syndrome is approximately 3 to 5 per 10,000 people. Brugada syndrome is approximately 8 to 10 times more common in males than females.
Brugada syndrome is also more prevalent in those who are of Southeast Asian descent.
The mean affected age is 41 years old.
Brugada syndrome accounts for 4% of all sudden cardiac deaths.
Symptoms of Brugada syndrome vary widely, with many individuals remaining asymptomatic.
Those who do experience symptoms might encounter blackouts, seizures, palpitations, chest discomfort, breathlessness, or dizziness.
Sudden cardiac death, often during sleep, is a potential outcome.
Brugada syndrome is usually diagnosed between 22 and 65 years of age It is rarely diagnosed in children.
Interestingly, symptoms might emerge due to triggers like high temperatures, alcohol intake, or dehydration.
The primary diagnostic tool is an ECG conducted in a hospital setting.
The use of high-lead positions is thought to increase the diagnostic yield by upto 1.5 times compared with standard lead positions.
When ECG findings remain equivocal or when spontaneous type 2 or type 3 Brugada pattern is observed, a drug challenge using sodium channel blockers is performed to evoke the type 1 Brugada ECG pattern.
Additionally, genetic testing for causative mutations should be considered in specific scenarios.
Three ECG pattern types were described in patients with Brugada syndrome, although only type 1 changes are considered diagnostic.
A type 1 Brugada ECG consists of coved ST-segment elevation greater than or equal to 2 mm with a negative T-wave in the right precordial leads, which are thought to be representative of pathophysiological changes in the RVOT.
Type 1 pattern consists of coved ST-segment elevation with T-wave inversion.
Type 2 and type 3 patterns consist of saddleback ST-segment configuration with variable levels of ST-segment elevation.
The ST-segment elevation is greater than 1mm in type 2 and less than 1 mm in type 3.
Pragmatically, only a type 1 pattern is diagnostic for Brugada syndrome, whereas patients with type 2 and type 3 patterns should undergo sodium channel blocker provocation testing.
Type 1 pattern consists of coved ST-segment elevation with T-wave inversion.
Type 2 and type 3 patterns consist of saddleback ST-segment configuration with variable levels of ST-segment elevation.
The ST-segment elevation is greater than 1mm in type 2 and less than 1 mm in type 3.
Brugada syndrome is diagnosed when type 1 Brugada ECG pattern is associated with one of the following clinical criteria:
Documented VF or polymorphic VT, Family history of sudden cardiac death at age younger than 45, Coved-type ECGs in family members, Inducibility of VT with programmed electrical stimulation, Syncope, or Nocturnal agonal respiration.
Unfortunately, there's no definitive cure for Brugada syndrome, but management options exist.
For high-risk cases, implantable cardioverter-defibrillators (ICDs) serve as a crucial intervention.
Additionally, pharmacological treatments like quinidine or novel therapies like radiofrequency ablation offer promise, although their efficacy might vary.
Note that, the only proven therapy is an implantable cardioverter-defibrillator (ICD).
Quinidine has been proposed as an alternative in settings where ICD’s are unavailable or where they would be inappropriate (for example, neonates).
High temperature, excessive alcohol consumption, and specific medications, can exacerbate symptoms.
Lifestyle modifications is important in Brugada syndrome to avoid triggers that provoke the condition.
In summary, the pathophysiology of Brugada syndrome involves a mutation in cardiac sodium channels.
Typical ECG shows a pseudo-RBBB and ST elevations in leads V1 and V2.
It is more prevalent in Asian males.
Treatment includes an implantable cardioverter-defibrillator to prevent sudden cardiac death.
Brugada syndrome is inherited in an autosomal dominant manner.
Brugada syndrome, though rare, demands careful management.
Personalized risk assessment, a multi-disciplinary approach, and regular follow-ups are pivotal.
While there's no cure, tailored interventions significantly reduce severe risks associated with this condition.
Brugada Syndrome
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