===== Brugada Syndrome =====

==== ECG – ST elevation V1,2,3 with RBBB. ====
  *autosomal dominant, most common gene SCN5A (20%) which encodes the Na+ channel
  *fibrotic changes are common, esp RV outflow tract
  *more in males, Asian
  *onset of symptoms – adulthood. Often after fever, after heavy meal, Xs alcohol
  *drugs which might exacerbate – flecainide, verapamil, propanolol, amitryptiline, alcohol, cocaine
  *most common rhythms – VF, polymorphic VT, AV re-entrant tachy
  *the most accepted theory to explain the ECG changes and the arrhythmogenic basis is based on the effect of the decrease of the inward positive currents (Na+, Ca+2) on the potassium transient outward current, whose expression levels vary across the myocardium layers (epicardium>endocardium)
  *<wrap em>A family history of sudden death does not translate into increased risk in relatives</wrap>

**3 forms. Only Type 1 ECG changes can confirm Dx of Brugada (T2,3 signs often seen in patients without synd):**

  - coved type ST elevation with at least 2mm (0.2mV) J-point  elevation and gradually descending ST segment followed by negative T-wave
  - has a saddle-back pattern with a least 2 mm J-point elevation and at least 1 mm ST elevation with a +ve or biphasic T-wave. Occasionally seen in healthy subjects.
  - either a coved (type 1 like) or a saddle-back (type 2 like) pattern, with less than 2 mm J-point elevation and less than 1 mm ST elevation. Not rare in healthy subjects.

{{:wiki:cardiovascular:brugadaecg.jpg?400|}}

=== This ECG abnormality must be associated with one of the following clinical criteria to make the diagnosis: ===

    *Documented ventricular fibrillation (VF) or polymorphic ventricular tachycardia (VT)
    *Family history of sudden cardiac death at <45 years old
    *Coved-type ECGs in family members
    *Inducibility of VT with programmed electrical stimulation
    *Syncope
    *Nocturnal agonal respiration

=== Management: ===

  * avoid risk factors - excess alcohol, certain drugs [[https://www.brugadadrugs.org/|Drug usage/avoidance in Brugada]], prompt treatment of fevers with anti-pyretics
  * quinidine sometimes. Isoprenaline infusion as emergency
  * implantable defib, catheter ablation
  * ECHO and MRI especially to exclude arrhythmogenic right ventricular cardiomyopathy
  * consider genetic testing SCN5A

[[https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-7/Management-of-patients-with-a-Brugada-ECG-pattern|Mx of Brugada ECG pattern]]\\ 
[[https://emedicine.medscape.com/article/163751-guidelines|EMed guidlelines]]\\ 
{{ :wiki:cardiovascular:ecg_conditions:anz_soc_of_cardiology_brugada_guidelines_2011.pdf |ANZ Soc of cardiologists - Brugada guidelines 2011}}

== Estimated 10% mortality per year from undiagnosed Brugada syndrome so admission for investigation probably should follow if Type 1 diagnosed. ==