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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
Which drugs should be avoided in Brugada syndrome?
Anti-arrhytmics Psychotropics Analgesics Anaesthetics
26
What is catecholaminergic polymorphic ventricular tachycardia (CPVT)?
Adrenergic induced di-directional and polymorphic VTs and SVT
27
What may trigger CPVT?
Emotional stress | Physical activity
28
What does ECG show in CPVT?
Nothing
29
What does echo show in CPVT?
Nothing
30
How is CPVT inherited?
AD or AR
31
Where is the mutation found in AD CPVT?
RyR2 | Ryanodine receptor mutation
32
Where is the mutation found in AR CPVT?
Cardiac calsequestrin gene (CASQ2)
33
What triggers may induce LQT interval in LQTS?
``` Exercise Loud noise (specifically in LQT2) ```
34
Family history is a major indicator of prognosis in Brugada syndrome true/false
False | No influence on prognosis
35
How is CPVT managed?
Everyone gets beta-blockers
36
What is Wolff-Parkinson-White syndrome?
Short PR interval
37
What characteristic ECG change is seen in WPW?
Delta wave
38
What is the follow up assessment for when a delta wave is seen on ECG?
To see how fast the accessory pathway in the heart works | If v fast, ablate
39
Where is the mutation in hypertrophic cardiomyopathy?
Sarcomeric genes
40
Which gender is more affected by dilated cardiomyopathy?
Males
41
Which gene is implicated in X-linked dilated cardiomyopathy?
Dystrophin
42
Where is the mutation in non-X-linked cardiomyopathy?
Sarcomeric and desmosomal genes
43
What characterises arrhythmogenic right ventricular cardiomyopathy? (ARCV)
Fibrofatty replacement of cardiomyocytes
44
Where is the mutation in AD ARCV?
Desmosomal proteins
45
Where is the mutation in AR ARVC?
Non-desmosomal genes
46
Which factors increase risk of cardiac sudden death in ARVC?
``` FHs Severity QRS prolongation VT Male ```
47
Where is the mutation in LQT1?
KCNQ1
48
Where is the mutation in LQT2?
KCNH2
49
Where is the mutation in LQT3?
SCN5a
50
Which drugs may cause LQT?
Amiodarone TCAs Citalopram/escitalopram Methadone
51
What does the SCN5a mutation do?
Alters function of sodium channels
52
How is Romano-Ward inherited?
AD
53
What is Anderson-Tawil syndrome?
LQT7
54
What are the clinical features of Anderson-Tawil syndrome?
Dysmorphic features VT Periods of paralysis
55
What are the clinical features of Timothy syndrome?
Small upper jaw Low set ears Flattened bridge of nose Syndactyly
56
What is Timothy syndrome?
LQT8
57
When does syncope occur in LQT1?
Exertion
58
When does syncope occur in LQT2?
Surprise/arousal | Post partum
59
When does syncope occur in LQT3?
At rest
60
Describe ECG findings in LQT1?
Broad based T wave
61
Describe ECG findings in LQT2
Low amplitude "notched" T wave
62
Describe ECG findings in LQT3
Late T waves
63
How is LQTS managed?
Asymp no FHx - monitor, lifestyle, beta blockers | Symp or strong FHs - ICD
64
What is affected in Brugada syndrome most commonly?
Na (v) 1.5 of sodium channel
65
When would ICD implantation be considered in Brugada syndrome?
Symptomatic patients
66
Outline pathogenesis in WPW?
Defect in AV node insulation leads to accessory pathways
67
Why is a delta wave present in (some) WPW?
Accessory pathway results in some early excitation of ventricles leading to a slurred upstroke - the delta wave
68
What is the most common tachycardia in WPW?
AV re-entrant
69
Which conditions is WPW associated with?
``` Ebstein's anomaly Coarctation of aorta VSD HCM Marfans Friedrich's ataxia ```
70
How does WPW present?
``` Palps Dizziness Dyspnoea Chest pain AF ```
71
What does a delta wave on ECG imply?
Left sided accessory pathway in WPW
72
How is WPW managed?
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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