Long QT

Question 1

Schwartz LQTS Score

Answer 1

• risk score to determine whether someone has long QT
• does the person have polymorphic VT? how long is the QT interval? How slow is the heart rate? Do they pass out with stress? etc
• you can have LQTS with a normal QT interval so this is problematic
• overlap between people with LQTS and normal individuals

Question 2

LQTS Genetics:

Answer 2

• 75% have known gene mutation
• novel or inherited (auto dom)
• location of channel increased risk (those in the transmembrane portion are more related with channel function and have greater risk)
• variable expressivity

Question 3

beta blockers

Answer 3

• decrease arrhythmias in LQTS
• 2 and 3 less protected than type 1
• decreases the amount of intracellular Ca

Question 4

Sudden death triggers in LQTS

Answer 4

• different triggers in different types
• Type 1: swimming
• Type 2: postpartum, auditory (alarm clock)
• Type 3: no pattern, clinical events more severe and less predictable

Question 5

Defibrillator therapy:

Answer 5

• lead screws into the heart muscle

- if heart rate gets too fast (ventricular defibrillation) it delivers pacing to stop it

Question 6

Polymorphic ventricular tachycardia

Answer 6

• fast heart rhythm in ventricle

- can cause asystole (death)