A 40 year old Asian man presents for a routine adult check-up and because his father, paternal grandfather, and paternal uncle all died of sudden cardiac death in their sleep before the age of 40, an EKG is taken. A shows a normal EKG. B shows this patient's EKG. The cardiac review of systems is notable for an episode of unexplained syncope a few months ago.Challenge: This is a rare cause of sudden cardiac arrest in a heart without a structural defect. What is it?
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Brugada syndrome
yay!
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Sodium Channelopathy
This is Brugada syndrome, associated with mutations in the SCN5A gene, which encodes the alpha subunit of the cardiac sodium channel. The EKG findings include a pseudo-RBBB and persistent ST segment elevation in V1-V3. In addition, the diagnosis requires one of the following: v-fib, v-tach, family history of sudden cardiac death at <45 years, ST elevation in family members, inducible VT, unexplained syncope suggestive of tachy-arrhythmia, or nocturnal agonal respiration. Brugada syndrome is related to life-threatening ventricular arrhythmias, usually between ages 22-65 and more common at night during sleep.
Sources: UpToDate; Wikipedia.
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