Genetic Arrythmias Flashcards
Long-QT syndrome is the most common familial arrhythmia linked to?
Sudden cardiac death
What is the main mutation of Long-QT syndrome?
KCNQ1 (K-channel)
What is the mechanism behind Long-QT syndrome?
Less depolarising (outward potassium) currents More depolarising (inward Na and Ca) currents
What are symptoms/signs of Long-QT syndrome?
Triggers like adrenaline or electrolyte disturbances can lead to polymorphic V-tach development
What are stimuli that can cause V-tach in Long-QT syndrome?
Sudden auditory stimuli
Physical/emotional stress
Swimming
During sleep
What finding on ECG indicates Long-QT syndrome?
QT prolongation > 440 ms in males
> 460 in females
besides ECG changes how is Long-QT syndrome diagnosed?
Presence of confirmed pathogenic mutation
How are low risk LQTS patients managed?
Lifestyle (avoidance)
Beta-blockers
How are high risk LQTS patients managed?
Lifestyle
ICD
+/- Beta blockers
What should be avoided in all LQTS patients?
QT-prolonging drugs
Brugada syndrome is caused by?
Cardiac sodium channel mutation (main SCNSA)
What can trigger VFib in Brugada syndrome?
Rest or sleep
Fever
Excessive alcohol
Large meals
What are signs/symptoms of brugada syndrome?
May present with LQTS before developing typical ECG cahnges
What are ECG findings of Brugada syndrome?
ST elevation and RBBB in V1-V3
Changes may be intermittent and change over time - may have t provocatively tested
How do you treat symptomatic Brugada syndrome?
ICD
