Congenital Long QT syndrome

Question 1

what is congenital long QT syndrome?

Answer 1

-a genetic disorder affecting the potassium voltage gated channels

Question 2

what causes congenital long QT syndrome?

Answer 2

autosomal dominant/recessive gene mutation

Question 3

what are some triggers for congenital long QT syndrome?

Answer 3

• exercise
• holding breath
• sudden auditory stimuli
• sleep
• QT prolongation states such as certain meds or hypokalaemia due to diarrhoea/vomiting

Question 4

symptoms of congenital long QT syndrome?

Answer 4

• syncope
• sudden death in children
• heart palpitations
• seizures

Question 5

what is shown on an ECG for long QT syndrome?

Answer 5

-QT prolongation
(>0.44s in males and >0.45s in females)
-Torsades de Pointes (polymorphic VT)

Question 6

management of congenital long QT syndrome?

Answer 6

• BB

- avoid QT prolongation drugs and triggers