Cardiac Na+ channelopathy, inherited autosomal dominant
Presentation: family history of sudden death, cardiac syncope (without exertion)
ECG: V1-3: ST elevation; biphasic V1 T wave
Diagnosis: infusion of ajmaline and rpt ECG
Management: implantable cardioverter-defibrillator (ICD)
Na+ channelopathy
First described by the 'Brugada Brothers' in 1992
History
Syncope without exertion
Family history of sudden death
Examination
Normal
Investigations
ECG: V1 & V2 ST elevation & T wave changes
Diagnostic criteria: ECG Brugada sign + clinical effect:
VF or VT
Family history of sudden death < 45yrs
Brugada sign on ECG of family members
Induced VT with electrical stimulation
Syncope
Nocturnal agonal respiration
Implantable cardio-defibrillator (ICD)
Can be Autosomal Dominant
Leads to syncope episodes usually from ventricular tachycardia
ECG
Varies, can be normal
Pseudo RBBB and TWI in V1
ST elevation in V1-3
Created in 2024.