Cardiac Arrhythmia and Sudden Cardiac Death Flashcards

1
Q

what is a sudden cardiac death in a young person?

A

event that is non-traumatic , non violent, unexpected and resulting from sudden cardiac arrest within 6 hours of previously witnessed normal health

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2
Q

causes of sudden cardiac death?

A
  • Inherited arrhythmia syndromes
    • Inherited cardiomyopathies
    • Inherited multi-system disorder (complicated with CVS issues)
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3
Q

examples of channelopathies?

A
  • Congenital long QT syndrome
    • Brugada syndrome
    • Catecholaminergic polymorphic ventricular tachycardia
    • Short QT syndrome
    • Progressive familial conduction disease
    • Familial AF
      Familial WPW
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4
Q

what is an after-depolarisation?

A
  • Abnormal depolarisations of cardiac myocytes that interrupt phase 2, 3 or 4 of the cardiac AP in the cardiac conduction system of the heart
    • Can lead to triggered activity seen as sustained cardiac arrhythmia
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5
Q

main implication of long QT?

A

long time taken for repolarisation means ore time for potential extra depolarisation

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6
Q

what are early after-depolarisations?

A

abnormal depolarisation during phase 2 or 3

caused by increase in frequency of abortive action potentials before normal repolarisation is complete

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7
Q

what causes early after-depolarisation in phase 2 and 3?

A

phase 2 = augmented opening of calcium channels

phase 3 = sodium channels

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8
Q

what can early after-depolarisations result in?

A

torsades de pointes

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9
Q

what can potentiate early after-depolarisations?

A
hypokalaemia 
drugs that prolong the QT interval 
- including class Ia and III anti-arrhythmics
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10
Q

risk of sudden death in congenital long QT?

A
  1. 33-0.9% annually

- risk associated with severity of prolongation

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11
Q

how is congenital long QT managed?

A
lifestyle (avoid strenuous exercise etc)
beta blockers
implanted defibrillator (only if risk of death >4%)
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12
Q

describe AD congenital long QT?

A

divided into 2

  • isolated long QT = romano ward syndrome
  • extra cardiac features = anderson-tawil syndrome, timothy syndrome
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13
Q

describe AR congenital long QT?

A

associated with deafness

AKA jervell and lange-nielsen syndrome

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14
Q

how is long QT diagnosed?

A

QT > 480ms on repeated ECG

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15
Q

describe LQT1?

A

most common type problem with potassium current

reduced function

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16
Q

LQT1 ECG?

A

check notes

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17
Q

describe LQT2?

A

2nd most common

also problem with potassium current

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18
Q

LQT2 ECG?

A

check notes

19
Q

describe LQT3?

A
problem with sodium current
increased function (problem)
20
Q

LQT3 ECG?

A

check notes

21
Q

there is a very low risk of sudden cardiac death in LQT, however what should be done to minimise risk further?

A

avoid drugs which prolong QT (e.g clarithromycin)
correct any electrolyte abnormality quickly
avoid strenuous exercise and loud noise in LQT2

22
Q

what is short QT?

A

mutation in K+ channels
rare
usually dont live long

23
Q

what is brugada syndrome?

A

AD genetic disorder causing abnormal electrical activity in the heart
risk of polymorphic VT and VF
atrial fibrilation is common

24
Q

ECG changes in brugada syndrome?

A

ST elevation and RBBB in V1-V3
- diagnostic ECG changes may only be seen with provocative testing with flecainide or ajmaline (drugs blocking sodium channels)

25
Q

genes associated with brugada syndrome?

A

12 associated

cardiac sodium channel (SCN5A) and calcium channel (CACN1Ac)

26
Q

how is brugada syndrome diagnosed?

A

ST elevation with type 1 morphology >2mm in one or more leads among the right precordial leads V1 and/or V2
can do after provocative drug test with sodium channel blockers

27
Q

what can trigger VF in brugada syndrome?

A

rest or sleep
fever
excessive alcohol or large meals
genotype and family history doesnt influence prognosis

28
Q

how is brugada managed?

A

avoid drugs causing ST elevation
avoid excessive alcohol or food
early paracetamol for fever (VERY IMPORTANT)
can use implantable defibrillator (if previous cardiac arrest)

29
Q

what is catecholaminergic polymorphic ventricular tachycardia?

A

Adrenergic induced bidirectional and polymorphic VT, SVTs, triggered by emotional stress and physical activity

30
Q

ECG/ECHO features of catecholaminergic polymorphic ventricular tachycardia?

A

both are normal

31
Q

what causes AD catecholaminergic polymorphic ventricular tachycardia?

A

ryanodine receptor mutation (RyR2)

32
Q

what causes AR catecholaminergic polymorphic ventricular tachycardia?

A

cardiac calsequestrin gene (CASQ2)

33
Q

management of catecholaminergic polymorphic ventricular tachycardia?

A

avoid competitive sport/stress etc
beta blockers
ICD implantation + beta blockers +/- flecainide if previous cardiac arrest or recurrent syncope

34
Q

what is wolf parkinson white syndrome?

A

congenital extra electrical pathway in the heart

35
Q

ECG features in wolf parkinson white?

A

short PR interval
delta wave
ventricular pre-excitation via (accessory pathway)

36
Q

what can WPW cause?

A

AVRT
AF
can cause sudden death
accessory pathway allows ventricles to fibrillate if atria fibrillate

37
Q

features of hypertrophic cardiomyopathy?

A

mutation in sarcomere genes
most people present with late heart failure rather than sudden death
1% CV mortality per year

38
Q

management of hypertrophic cardiomyopathy?

A

avoid competitive sport

ICD if previous cardiac arrest

39
Q

what happens in dilated cardiomyopathy?

A

problem somewhere in proteins (sarcomeres) causing dilation of heart muscle over time

40
Q

what happens in arrhythmogenic right ventricular cardiomyopathy?

A

fibro-fatty replacement of cardiomyocytes

LV involvement in >50% of cases

41
Q

what causes arrhythmogenic right ventricular cardiomyopathy?

A

AD mutations in the genes for autosomal proteins

AR mutation in non-desmosomal genes

42
Q

what is associated with increased risk of sudden death in arrhythmogenic right ventricular cardiomyopathy?

A
family history of sudden death 
severe right/left ventricle dysfunction
frequent non-sustained VT
QRS prolongation
VT induction on EPS
male
43
Q

management of arrhythmogenic right ventricular cardiomyopathy?

A
avoid strenuous exercise
beta blockers
ICD implantation
amiodarone considered in people who cant have beta blockers
can have catheter ablation