Channelopathies Flashcards

1
Q

What is Brugada syndrome?

A

Autosomal dominant arrythmogenic disorder of Na? channels in heart that can predispose to VFib and death

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2
Q

What is the clinical presentation of Brugada syndrome?

A
  • some pts are asymptomatic for life
  • some just have syncope (due to VFib)
  • some just drop dead
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3
Q

What is the typical ECG of Brugada and how to diagnose?

A

-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

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4
Q

What if they have a suspicious history but their ECG is indeterminate for Brugada?

A

-drug challenge test with Na channel blocker

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5
Q

Management of brugada syndrome?

A
  • ICD for symptomatic pts (hx of syncope/VtachVFib episodes)
  • meds to prevent Vfib (isoproterenol, quinidine)
  • dont clear them for exercise or duty
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