Channelopathies Flashcards
What is Brugada syndrome?
Autosomal dominant arrythmogenic disorder of Na? channels in heart that can predispose to VFib and death
What is the clinical presentation of Brugada syndrome?
- some pts are asymptomatic for life
- some just have syncope (due to VFib)
- some just drop dead
What is the typical ECG of Brugada and how to diagnose?
-typical ECG has coved-type ST elevation and negative T wave in right pre-cordial leads (V1/V2)
-diagnosis consists of 1) typical ECG, 2) clinical characteristics, to include;
(1) a history of VT/VF
(2) a family history of SCD
(3) a family history of
coved-type ECG
(4) agonal respiration during sleep
(5)inducibility of VT/VF during electrophysiological study
What if they have a suspicious history but their ECG is indeterminate for Brugada?
-drug challenge test with Na channel blocker
Management of brugada syndrome?
- ICD for symptomatic pts (hx of syncope/VtachVFib episodes)
- meds to prevent Vfib (isoproterenol, quinidine)
- dont clear them for exercise or duty