33 male-smoker, denies cocaine use or family history of sudden cardiac death, presents to the ED with dry cough, chills, myalgias x 1 week now complicated by one episode of syncope one-night prior without any chest pain, palpitations, SOB, incontinence. After having LOC, he awoke, crawled into bed and went to sleep. The next day his girlfriend noticed a bruised left eye and urged him to come to the ED.

EKG done at triage is below, what is the diagnoses???

Brugada is a channelopathy, a genetic disorder of a cardiac channel, most commonly associated with sodium channel disruption leading to arrythmias and sudden cardiac death.

annual risk of SCD in general population 1/10,000
annual risk of SCD in Brugada population 300/10,000

*SCD = sudden cardiac death

These are the 3 types…

File:Brugada.jpg

 

Type 1 is diagnostic of Brugada syndrome.

Definitive diagnoses is made by…

Type 1 in at least one precordial lead (V1 to V3)  with one of the following…

  • episode of Vfib/VTach
  • family history of SCD < 45
  • Type 1 noted in family members
  • induced VTach in the setting of electrical stimulation, syncope, or nocturnal agonal respiration (reports of SCD in SE asians occuring at bedtime found to be Brugada)

OR

  • baseline of type 2 or type 3 that then transforms into type 1 when stressed by sodium channel blockers

 

Patients with syncope and type 1 Brugada, with no other known cause, will require an ICD.

Patients with incidental type 1, 2 or 3 should be discussed with cardiology.

References

  1. PMID 19606473 (genetics)
  2. PMID: 15655131 (2005 circulation)
  3. PMID: 12417552 (2002 circulation)