arrythmia stuff Flashcards
RWD syndrome mutation
- in the slow cardiac K+ channel IKs (LQT1),
- the rapid cardiac K+ channel IKr (LQT2)
- the cardiac Na+ channel INa (LQT3).
JLNS mutations are
homozygous carriers of mutations in IKs (LQT1)
JLNS symptoms
congenital deafness, (homozygous)
while the heterozygous carriers are asymptomatic
the result of reducing the number of K+ channels expressed in the myocyte plasma membrane is?
reduces the size of the K+ current (IKr + IKs) that helps terminate the plateau phase of the fast response and return the membrane to resting potential during diastole.
aka. prolonged plateau
effect of LQT1 mutant slow K+ channels
reduced current amplitude
effect of LQT3 mutant na+ channels
incomplete activation
brugada syndrome mutations
- More than 30 different mutations in the cardiac Na+ channel have been linked to Brugada,
- with many of these reducing peak inward Na+ current that drives action potential upstroke in ventricular myocytes.
triggered afterdepolarizations: triggered by
action potentials
Fast response phase 2 is prolonged by
- ↑ inward current
(incomplete Na+ channel inactivation in LQT3) or by - ↓ outward current
(↓ K+ current in LQT1, LQT2).
Ca2+ entry during the prolonged QT interval triggers
- EADs
(via Ca2+ channel reactivation) or - DADs
(via NCX-dependent depolarization).
↑ sympathetic tone (startle) ↑ likelihood of
triggered afterdepolarizations because Ca2+ influx is enhanced by β-adrenergic receptor activity.
heart failure increases the
frequency of occurrence of triggered afterdepolarizations (even without LQT mutations).
Amiodarone is a class
III drug that has, important for its utility, class I action too.
• Class I drugs slow
upstroke
• Class Ib drugs show
pure class I action: slow upstroke, decreased AP duration