Cardiac Conditions that Cause Sudden Death Flashcards

1
Q

Define sudden cardiac death

A

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

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2
Q

Is Brugada syndrome a channelopathy or cardiomyopathy?

A

Channelopathy

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3
Q

What is a channelopathy?

A

Ion current imbalance + development of early/late depolarizations

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4
Q

What is a cardiomyopathy?

A

Arrhythmogenesis related to scar/electrical barrier formation and subsequent re-entry

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5
Q

Are channelopathies normal or abnormal at screening?

A

Often normal

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6
Q

Are cardiomyopathies normal or abnormal at screening?

A

Often abnormal

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7
Q

What are the mechanisms of arrhythmia?

A

Automaticity - thing that keeps node firing
Trigger
Re-entry e.g. scar

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8
Q

When do ‘after depolarizations’ occur?

A

interrupt phase 2,3 or 4 of cardiac action potential

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9
Q

What do after depolarizations lead to?

A

Triggered activity - sustained cardiac arrhythmia e.g. fibrillation

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10
Q

When do early after depolarizations occur?

A

During phase 2 or 3

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11
Q

What happens during phase 4 of the cardiac action potential?

A

Repolarization phase

Ventricular myocyte at rest

K+ channels open

Resting membrane potential = -90mV

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12
Q

What happens during phase 0 of the cardiac action potential and why?

A

Fast Na+ influx

Depolarization

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13
Q

What happens during phase 1 of the action potential cardiac cycle?

A

Closure of Na+ channels and transient K+ efflux

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14
Q

What happens during phase 2 of the action potential cycle?

A

Ca++ influx

Plateau

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15
Q

What happens during phase 3 of the cardiac action potential cycle?

A

Closure of Ca++ channels and K+ efflux

Rapid repolarization

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16
Q

What type of activity do after depolarizations lead to?

A

Triggered activity - sustained cardiac arrhythmia

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17
Q

What causes an early after depolarization?

A

Increase in the frequencyof abortive action potentials before normal repolarization is complete

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18
Q

Why might phase 2 be interrupted by an early afterdepolarization?

A

Augmented opening of Ca2+ channels

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19
Q

Why might phase 3 be interrupted by an early afterdepolarization?

A

Opening of Na2+ channels

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20
Q

What do early afterdepolarizations cause?

A

Torsades de Pointes

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21
Q

What is Torsades de Pointes?

A

Specific form of VT in patients with long QT syndrome.

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22
Q

What might Torsades de Pointes look like on ECG?

A

Rapid, irregular QRS complexes

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23
Q

What can cause early afterdepolarizations?

A

Hypokalemia

Drugs that prolong QT interval

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24
Q

Are the vast majority of cardiac conditions dominantly or recessively inherited?

A

Dominantly

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25
When do delayed afterdepolarizations occur?
Phase 4 after repolarization complete but before another action potential would normally occur
26
What causes delayed afterdepolarizations?
Elevated cytosolic Ca2+ concentrations
27
What drug toxicity can cause elevated cytosolic Ca2+ concentrations?
Digoxin
28
What causes the delayed afterdepolarization?
Overload of sarcoplasmic reticulum causes spontaeous Ca2+ release after repolarization Causes relased Ca2_ to exit cell through 2Na+/2Ca2+ exchanger Results in net depolarizing current
29
Bidirectional ventricular tachycardia is a classical feature of what afterdepolarization?
Delayed afterdepolarization | also catecholaminergic polymorphic ventricular tachycardia/CPVT
30
What causes QT prolongation?
Less repolarising current prolongs APD (action potential duration) OR More depolarising current prolongs APD
31
Polymorphic VT is triggered by what in congenital LQTS?
Adrenergic stimulation
32
What is the name of the autosomol dominant congenital LQT syndrome that causes isolated LQT?
Romano-Ward syndrome
33
What is Jervell and Lange-Nielsen-syndrome? Is it autosomal dominant or recessive?
Long QT syndrome associated with deafness Autosomal recessive
34
Are you likely to die from LQT syndrome?
No
35
What is the treatment for long QT syndrome?
Beta blockers
36
What should people with LQTS1 avoid?
Strenuous swimming
37
What should people with LQTS2 avoid?
Exposure to loud noises
38
How is long QT syndrome diagnosed?
QTc >480ms in repeated 12-lead ECG or LQTS risk score >3
39
What criteria is used to diagnose LQT syndrome?
Schwartz criteria
40
What is the criteria for diagnosing congenital short QT syndrome?
QT < 300ms at HR < 80bpm
41
Congenital short QT syndrome may be associated with what other cardiac condition?
AF
42
Are you likely to die from congenital short QT syndrome?
Yes
43
What are some of the problems of implanting a cardioverter defibrillator in children?
Body size Psychological Inappropriate discharges due to T wave oversensing
44
What drugs could you give in congenital short QT syndrome?
Anti-arrhythmic drugs e.g. quinidine, flecainide
45
What condition causes RBBB in V1-3 and ST elevation?
Brugada syndrome
46
A patient who has brugada syndrome has an increased risk of developing what arrhythmias?
VT | VF
47
ECG changes in Brugada syndrome may be intermittent. How can you get a diagnostic ECG in a patient who may have Brugada syndrome?
Provocative testing with flecainide or ajmaline | drugs that block the cardiac Na channel
48
Why are flecainide and ajmaline used in the provocative testing of a patient with Brugada syndrome?
Block the cardiac sodium channel
49
What 2 gene mutations are associated with Brugada syndrome?
SCN5A - sodium channel | CACN1Ac - calcium channel
50
What is the pathophysiology of Brugada syndrome?
Channelopathy Alters transmembrane ion currents SCN5A gene codes for cardiac voltage gated Na channel Defect causes reduced Na current available during phase 0 and early repolarization of cardiac potential
51
Is Brugada syndrome inheritance mostly dominant or recessive?
Dominant
52
After provocative drug testing, what would you see on the ECG of a patient with Brugada syndrome?
ST segment elevation with type 1 morphology in 1 or leads (V1 and/or V2)
53
What can trigger VF in Brugada syndrome?
Rest/sleep Fever Excessive alcohol Large meals
54
What lifestyle changes should patients with Brugada syndrome make?
Avoid drugs that induce ST segment elevation in right precordial leads Avoid excessive alcohol + large meals Prompt treatment of fever
55
When would you consider implanting an ICD in a patient with Brugada syndrome?
Previous cardiac arrest Documented sponatenous sustained VT
56
What drugs would you avoid in Brugada syndrome?
Anti-arrhythmics Psychotropics Analgesics Anaesthetics
57
What condition is described: Adrenergic induced bidirectional and polymporphic VT, SVTs?
Catecholaminergic polymorphic ventricular tachycardia
58
What triggers catecholaminergic polymorphic ventricular tachycardia?
Emotional stress | Physical activity
59
What can be seen on a patient's echo and ECG in catecholaminergic polymorphic ventricular tachycardia?
Nothing | Normal
60
How is catecholaminergic polymorphic ventricular tachycardia investigated?
Autosominal dominant OR recessive (wheyyyyyy u fooled)
61
What gene mutation is associated with dominant inheritance of catecholaminergic polymorphic ventricular tachycardia (CPVT)?
Ryanodine Receptor mutation (RyR2)
62
What gene mutatio nis associated with recessive inheritance of catecholaminergic polymorphic ventricular tachycardia?
Cardiac calsequestrin gene (CASQ2)
63
What lifestyle changes are recommended for patients with CPVT?
Avoidance of competitive sports, strenuous exercise + stressful environments
64
A patient has recently been diagnosed with CPVT. What drug should they be put on?
Beta blockers
65
When should you implant an ICD in a patient with CPVT?
If they've experienced cardiac arrest, recurrent syncope or polymorphic/bidirectional VT despite optimal therapy
66
Beta blockers should be considered for genetically positive family members of a person with CPVT despite a negative exercise test. True or false?
True
67
What should be considered in addition to beta blockers in patients with CPVT that have had recurrent syncope or polymorphic/bidirectional VT whilst on beta blockers (who are not suitable for an ICD)?
Flecainide
68
What is a last resort therapy for CPVT after drugs and ICD?
left cardiac sympathetic denervation
69
What is the pathophysiology of Wolff-Parkinson-White syndrome (WPW)?
Presence of accessory pathway between atrium and ventricle
70
AVRT (atrioventricular re-entrant tachycardia) is the most common arrhythmia in what condition?
WPW
71
Squatting babies are associated with what cardiac condition?
Tetralogy of fallot
72
What are the 4 components of tetralogy of fallot?
Pulmonary stenosis Ventricular septal defect Right Ventricular hypertrophy Overriding aorta
73
A mutation in what genes causes hypertrophic cardiomyopathy?
Sarcomeric genes (MYH7/MYBPC3)
74
How does hypertrophic cardiomyopathy tend to present? (4)
Sudden death Heart failure End stage heart failure AF
75
What is the treatment for hypertrophic cardiomyopathy?
ICD if severe (prior cardiac arrest/sustained VT) or lotsa risk factors (FHx in first degree relative, LV wall thickness >30mm, recent unexplained syncope)
76
What can you use if you are unsure of a patient's risk of sudden cardiac death?
HOCM: SCD risk calculator
77
What lifestyle modifications should people with HOCM make?
Avoid competitive sports
78
What genes cause dilated cardiomyopathy?
Sacromere + desmosomal genes laminA/C and desmin if conduction disease Dystrophin if X-linked
79
Who tends to get dilated cardiomyopathy, males or females?
Males | not common in childhood
80
Describe the pathogenesis of arrhythmogenic right ventricular cardiomyopathy
Fibro-fatty replacement of cardiomyocytes
81
What autosomal dominnat mutations cause arrythmogenic right ventricular cardiomyopathy?
Desmosomal proteins
82
What autosomal recessive mutations cause arrhythmogenic right ventricular cardiomyopathy?
Nondesmosomal genes
83
What arrhythmia can be caused by arrhythmogenic right ventricular cardiomyopathy?
VT
84
What lifestyle modifications are recommended in arrhythmogenic right ventricular cardiomyopathy?
Avoidance of competitive sports
85
What is the first line therapy in arrhythmogenic right ventricular cardiomyopathy?
Beta blockers
86
What drug can be used in patients intolerant of beta blockers in arrhythmogenic right ventricular cardiomyopathy?
Amiodarone
87
Is ICD used in arrhythmogenic right ventricular cardiomyopathy?
YEah In patients with history of aborted SCD and haemodynamiclaly poorly tolerated VT
88
What is the only known treatment for VF?
Defibrillation
89
What does defibrillation do?
Delivers high energy to all cardiac myocytes causing them to fully depolarise (resets them)
90
Lamin A/C mutations are associated with which inherited condition?
Dilated cardiomyopathy First degree heart block SVT and VA
91
What is the treatment for all patients with DCm and confirmed disease-causing LMNA mutation and risk factors?
ICD