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…
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
- PMID 19606473 (genetics)
- PMID: 15655131 (2005 circulation)
- PMID: 12417552 (2002 circulation)