Related Subjects:
| Brugada Syndrome
| Long QT syndrome (LQTS) Acquired
| Long QT syndrome (LQTS) Congenital
| Torsades de Pointes
| Ventricular Fibrillation
| Ventricular Tachycardia
| Resuscitation - Adult Tachycardia Algorithm
| Automatic Implantable Cardioverter Defibrillator (AICD)
The only known effective
treatment is an implantable defibrillator
About
- Brugada syndrome is a channelopathy
- A cause of sudden cardiac death with distinctive ECG changes
- Common - Affects 1-6 per 1000 especially SE Asian Men
History
- Brugada was discovered by two Spanish brothers – cardiologists Pedro and Josep Brugada.
- The genetic work was done by their brother Ramon.
Aetiology
- Higher frequency (1/2500) may be found in eastern countries, especially Thailand, where Brugada is considered the major cause of sudden death in young individuals
- Up to 30% have loss of function mutations in the sodium channel SCN5A gene (Chr 3).
- Men x 8 incidence
Clinical
- Sudden Unexplained Nocturnal Death Syndrome
- Syncope and cardiac arrest
Investigations
- FBC, U&E etc all normal
- Structure: The heart is at least macroscopically structurally normal and so CXR, Echo, Cardiac MRI, catheterization, and myocardial biopsy findings are normal
- Prolonged telemetry/ 24 hr tape or longer may show VT/TDP
- ECG shows ST segment elevation in leads V1 to V3. The QT interval is usually normal. Incomplete or complete right bundle branch block. Polymorphic ventricular tachycardia and VF. Challenge with sodium channel blockers is positive when the drug generates a J wave with an absolute amplitude of 2 mm or more in leads V1, V2, and/or V3 with or without an RBBB.
- Genetically testing for a mutation in SCN5A
- Electrophysiological studies
ECG/EKG
Risk factors for SCD include
- Aborted SCD
- ventricular arrhythmias
- Spontaneous ST elevation
- Inducible VT/VF
- Syncope
Management
- Avoid High fevers, excess alcohol , large meals, Cocaine, Alpha blockers, TCAs, First generation antihistamines, Flecainide, propafenone, procainamide, disopyramide
- Avoid harmful drugs - see Safe drugs in Brugada website
- Idiopathic VF - sudden death is the main concern. No drug treatment for Brugada syndrome is recommended. ICD is the treatment of choice for those with an inducible arrhythmia on EPS. ICD may be considered in high risk asymptomatic family members but is controversial
- Quinidine has been useful for some. Catheter ablation of the RVOT has been considered for some with recurrent shocks
References