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Year - 3 Sudden Death > SCD Conditions > Flashcards

SCD Conditions Flashcards

1
Q

Outline the pathogenesis of arrhythmogenic channelopathies

A

Ion current imbalance

Development of early and late depols

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2
Q

Outline the pathogenesis of arrhythmia related to cardiomyopathies

A

Scar/electrical barrier formation and subsequent re-entry

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3
Q

What are afterdepolarisations?

A

Abnormal depols which interrupt phase 2, 3 or 4 of cardiac AP

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4
Q

When does an early afterdepolarisation occur?

A

In phase 2 and 3

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5
Q

What causes an early afterdepolarisation?

A

Increase in freq of APs before normal repolarisation

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6
Q

Which channels open to interrupt phase 2?

A

Calcium

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7
Q

Which channels open to interrupt phase 3?

A

Sodium

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8
Q

When do delayed afterdepolarisations occur?

A

Phase 4

After repol complete but before another AP

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9
Q

What causes a delayed afterdepolarisation?

A

Elevated cytosolic calcium conc

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10
Q

What is Romano-Ward syndrome?

A

Isolated long QT

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11
Q

What is AR LQTS associated with?

A

Sensorineural deafness

Jervell and Lange-Nielsen syndrome

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12
Q

What are Anderson-Tawil and Timothy syndrome?

A

Types of AD LQTS with extra cardiac features

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13
Q

Outline the molecular pathology in LQTS

A

Reduced or dysfunctional ionic current
Prolonged repol
QT prolongation

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14
Q

Describe the ECG appearance in LQTS?

A

Polymorphic VT

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15
Q

Who is most at risk of sudden cardiac death in LQTS?

A

Pre-adolescent males
Adults females
Long/increasing QT duration

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16
Q

Which drugs should be avoided in LQTS?

A

Clarithromycin
Azithromycin
Anaesthetics

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17
Q

What causes congenital short QT syndrome?

A

Mutated K+ channels

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18
Q

How is Romano-Ward syndrome inherited?

A

AD

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19
Q

Which other heart condition is congenital short QT associated with?

A

AF

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20
Q

What does ECG show in brugada syndrome?

A

ST elevation and RBBB in V1-V3

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21
Q

Which drugs can assist in diagnosis of Brugada syndrome?

A

Flecainide

Ajmaline

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22
Q

Which main genes are associated with Brugada syndrome?

A

SCN5a

CACN1Ac

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23
Q

How is Brugada syndrome inherited?

A

AD

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24
Q

What may trigger VF in Brugada syndrome?

A

Rest/sleep
Fever
Excessive alcohol
Large meals

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25
Q

Which drugs should be avoided in Brugada syndrome?

A

Anti-arrhytmics
Psychotropics
Analgesics
Anaesthetics

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26
Q

What is catecholaminergic polymorphic ventricular tachycardia (CPVT)?

A

Adrenergic induced di-directional and polymorphic VTs and SVT

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27
Q

What may trigger CPVT?

A

Emotional stress

Physical activity

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28
Q

What does ECG show in CPVT?

A

Nothing

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29
Q

What does echo show in CPVT?

A

Nothing

30
Q

How is CPVT inherited?

A

AD or AR

31
Q

Where is the mutation found in AD CPVT?

A

RyR2

Ryanodine receptor mutation

32
Q

Where is the mutation found in AR CPVT?

A

Cardiac calsequestrin gene (CASQ2)

33
Q

What triggers may induce LQT interval in LQTS?

A 
Exercise
Loud noise (specifically in LQT2)
34
Q

Family history is a major indicator of prognosis in Brugada syndrome
true/false

A

False

No influence on prognosis

35
Q

How is CPVT managed?

A

Everyone gets beta-blockers

36
Q

What is Wolff-Parkinson-White syndrome?

A

Short PR interval

37
Q

What characteristic ECG change is seen in WPW?

A

Delta wave

38
Q

What is the follow up assessment for when a delta wave is seen on ECG?

A

To see how fast the accessory pathway in the heart works

If v fast, ablate

39
Q

Where is the mutation in hypertrophic cardiomyopathy?

A

Sarcomeric genes

40
Q

Which gender is more affected by dilated cardiomyopathy?

A

Males

41
Q

Which gene is implicated in X-linked dilated cardiomyopathy?

A

Dystrophin

42
Q

Where is the mutation in non-X-linked cardiomyopathy?

A

Sarcomeric and desmosomal genes

43
Q

What characterises arrhythmogenic right ventricular cardiomyopathy? (ARCV)

A

Fibrofatty replacement of cardiomyocytes

44
Q

Where is the mutation in AD ARCV?

A

Desmosomal proteins

45
Q

Where is the mutation in AR ARVC?

A

Non-desmosomal genes

46
Q

Which factors increase risk of cardiac sudden death in ARVC?

A 
FHs
Severity
QRS prolongation
VT
Male
47
Q

Where is the mutation in LQT1?

A

KCNQ1

48
Q

Where is the mutation in LQT2?

A

KCNH2

49
Q

Where is the mutation in LQT3?

A

SCN5a

50
Q

Which drugs may cause LQT?

A

Amiodarone
TCAs
Citalopram/escitalopram
Methadone

51
Q

What does the SCN5a mutation do?

A

Alters function of sodium channels

52
Q

How is Romano-Ward inherited?

A

AD

53
Q

What is Anderson-Tawil syndrome?

A

LQT7

54
Q

What are the clinical features of Anderson-Tawil syndrome?

A

Dysmorphic features
VT
Periods of paralysis

55
Q

What are the clinical features of Timothy syndrome?

A

Small upper jaw
Low set ears
Flattened bridge of nose
Syndactyly

56
Q

What is Timothy syndrome?

A

LQT8

57
Q

When does syncope occur in LQT1?

A

Exertion

58
Q

When does syncope occur in LQT2?

A

Surprise/arousal

Post partum

59
Q

When does syncope occur in LQT3?

A

At rest

60
Q

Describe ECG findings in LQT1?

A

Broad based T wave

61
Q

Describe ECG findings in LQT2

A

Low amplitude “notched” T wave

62
Q

Describe ECG findings in LQT3

A

Late T waves

63
Q

How is LQTS managed?

A

Asymp no FHx - monitor, lifestyle, beta blockers

Symp or strong FHs - ICD

64
Q

What is affected in Brugada syndrome most commonly?

A

Na (v) 1.5 of sodium channel

65
Q

When would ICD implantation be considered in Brugada syndrome?

A

Symptomatic patients

66
Q

Outline pathogenesis in WPW?

A

Defect in AV node insulation leads to accessory pathways

67
Q

Why is a delta wave present in (some) WPW?

A

Accessory pathway results in some early excitation of ventricles leading to a slurred upstroke - the delta wave

68
Q

What is the most common tachycardia in WPW?

A

AV re-entrant

69
Q

Which conditions is WPW associated with?

A 
Ebstein's anomaly
Coarctation of aorta
VSD
HCM
Marfans
Friedrich's ataxia
70
Q

How does WPW present?

A 
Palps
Dizziness
Dyspnoea
Chest pain
AF
71
Q

What does a delta wave on ECG imply?

A

Left sided accessory pathway in WPW

72
Q

How is WPW managed?

A

Asymp: monitor and considered ablation
Symp: ablation
Acute: DC cardioversion (unstable) or anti-arrhythmics (stable)

Year - 3 Sudden Death (4 decks)

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