Brugada syndrome is an autosomal dominant disorder; most commonly due to sodium channel gene mutation; characterized by the ECG findings of a RBBB and ST segment elevations in the right precordial leads (V1-V3).
Presents with ventricular tachycardia, ventricular fibrillation and sudden cardiac death.
Criteria for Brugada syndrome:-
1. Pattern 1 on ECG (≥2 leads:V1/V2/V3); spontaneous or with drug challenge (sodium channel blocking agent), OR
2. Pattern 2 or 3 (≥2 leads:V1/V2/V3) with conversion to pattern 1 with drug challenge.
Pattern 1 on ECG/Pattern 2 or 3 along with≥ following features:-
1. Documented ventricular fibrillation (VF) or polymorphic ventricular tachycardia (VT).
2. Family history of SCD at < 45 years old.
3. Pattern 1 ECG in the family members.
4. VT during programmed stimulation.
5. Syncope.
6. Nocturnal agonal respiration.
1. Documented ventricular fibrillation (VF) or polymorphic ventricular tachycardia (VT).
2. Family history of SCD at < 45 years old.
3. Pattern 1 ECG in the family members.
4. VT during programmed stimulation.
5. Syncope.
6. Nocturnal agonal respiration.
ECG shows coved ST elevations > 2mm accompanied with an inverted T wave in leads V1-V3 (pattern 1), >2mm of saddleback shaped ST elevation in leads V1-V3 (pattern 2) and <2 mm of saddleback shaped ST elevation in leads V1-V3 (pattern 3). This pattern may be present chronically or intermittently; In this later case, the syndrome may be unmasked by administering sodium channel blocking antiarrhythmic drugs (e.g. procainamide, flecainide).
Treatment:- Implantable cardioverter defibrillator (ICD) is the mainstay of treatment.
Figure:- ECG showing pattern 1, 2 and 3 of brugada syndrome.
Figure:- ECG showing pattern 1, 2 and 3 of brugada syndrome.
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