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Year 3 > Sudden Death > Flashcards

Sudden Death Flashcards

1
Q

3 inherited cardiac causes of sudden death?

A

Inherited arrhythmia syndrome

Inherited cardiomyopathy

Inherited multi system disease with CVS involvement, e.g. myotonic dystrophy

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2
Q

Another name for arrhythmia syndromes?

A

Channelopathies

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3
Q

6 inherited channelopathies

A

Congenital LQTS

Brugada syndrome

Catecholaminergic polymorphic VT

Short QT syndrome

Familial AF

WPW

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4
Q

An inherited cardiomyopathy

A

hypertrophic cardiomyopathy

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5
Q

Screening is routinely offered for which type of cardiomyopathy?

A

Dilated

Hypertrophic

Arrhythmogenic right ventricular cardiomyopathy

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6
Q

When do early depolarisations occur?
What arrhythmia do they cause?
What Potentiates them?

A

Phases 2 and 3 of cardiac AP

Torsades de Pointes

Hypokalaemia and Class Ia and III anti-arrhythmatics which prolong QT interval

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7
Q

When do delayed depolarisations occur?
What inherited condition are they seen in?
What arrhythmia do they cause?

A

phase 4 of cardiac AP

Catecholaminergic polymorphic ventricular tachycardia

Bidirectional VT

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8
Q

Inheritance pattern of cardiac conditions?

A

AD

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9
Q

Most common inherited cardiac cause of sudden death?

How many people are carriers for the gene?

A

LQTS

1 in 2000

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10
Q

What arrhythmia is caused by LQTS?

What triggers the arrhythmia’s?

A

Torsades de Pointes
(polymorphic VT)

Triggered by adrenergic stimulation, e.g. excitement, exercise

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11
Q

What is Romano Ward Syndrome?

Inheritance?

A

Most common form of LQTS

AD

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12
Q

What LQTS is assoc w deafness?

Inheritance?

A

Jervell and Lange-Nielsen Syndrome

AR

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13
Q

Pathophysiology behind LQTS?

A

Reduced ionic current = prolonged repolarisation = QT interval prolongation

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14
Q

How is LQTS diagnosed?

A

QT>480ms (>0.48s/>2 big boxes and 2 small boxes)

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15
Q

Symptoms of LQTS?

A

Syncope
Stress
Potentially HX of deafness (JALNS)

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16
Q

5 meds that should be avoided in LQTS?

A 
Clarithromycin
Azithromycin
Imipramine
Amitriptyline
Haloperidol
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17
Q

LQTS1 prophylactic advice?

A

Avoid strenuous exercise

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18
Q

LQTS2 prophylactic advice?

A

Avoid loud noises

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19
Q

What is affected in SQTS (short QT)?
How long does it have to be for this?
Who’s is affected by SQTS?

A

K+ channels

<300ms (less than 1 big box and 3 small boxes) at HR <80bpm

Young children

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20
Q

SQTS puts you at risk of what arrhythmia?

A

TdP (polymorphic VT)

VF

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21
Q

What arrhythmia do people with Brugada syndrome get?

A

AF

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22
Q

ECG abnormalities in Brugada syndrome?

A

ST elevation and RBBB affecting V1-V3

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23
Q

Inheritance of Brugada syndrome?

Who gets it?

A

AD

Young males

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24
Q

Triggers for VF in Brugada Syndrome?

A

Usually sleep or rest
Fever
Excess alcohol
Large meals

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25
Q

Channels and genes assoc w Brugada syndrome?

A

Cardiac sodium channel (SNC5A)

Calcium channel (CACN1Ac)

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26
Q

Treatment of Brugada Syndrome?

A

Avoid triggers
Avoid Na channel blocker/anti-arrhythmatics
Go into hospital if fever
ICD if severe

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27
Q

What is catecholaminergic polymorphic ventricular tachycardia?
Triggers?

A

adrenergic-induced bidirectional and polymorphic VT and SVT’s

Stress (e.g. alarm clocks, jumping in cold pool), physical activity

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28
Q

ECG in CPVT?

A

usually normal unless induced arrhythmia, echo also normal

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29
Q

Autosomal dominant CPVT gene?

Autosomal recessive CPVT gene?

A

Dominant - Ryanodine receptor mutation (RyR2)

Recessive - Cardiac Calsequestrin gene (CASQ2)

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30
Q

Treatment of CPVT?

Prophylaxis?

A

Defibrillate if necessary

Prophylaxis:

  • avoid triggers
  • B-blockers
  • +/- Flecainide
  • +/- ICD
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31
Q

3 ECG features of WPW?

A

Short PR
Delta waves
Ventricular pre-excitation

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32
Q

Most common arrhythmias in WPW?

A

AV re-entry tachycardia

AF

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33
Q

Pathophysiology of WPW?

A

No conduction delay between atria and ventricles due to an accessory pathway bypassing AV node

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34
Q

Treatment for WPW?

A

Leave it if accessory pathway does not conduct quickly

Otherwise, ablate the pathway

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35
Q

Most common cardiomyopathy?

What type of mutation is most common? What gene is affected most commonly?

A

Hypertrophic

Sarcomere gene mutation - most common MYBPC3

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36
Q

What % of patients with HOCM die each year?

A

1%

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37
Q

2 ECG changes that suggest hypertrophy?

A

Positive deflection in V5

Negative deflection in V2

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38
Q

Treatment of HOCM?

A

ICD if arrest, sustained VT or high risk e.g. 1st degree FHx, thick wall, recent syncope

If none then leave it as risk of cardiac event <4%

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39
Q

Who gets dilated cardiomyopathy?

A

adulthood-elderly

Men

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40
Q

Genes and structures related to dilated cardiomyopathy?

A

Sarcomere and desmosomal genes

Lamina A/C if there is conduction disease

Dystrophin gene if X-linked (muscular dystrophy)

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41
Q

Pathophysiology of arrhythmogenic right ventricular cardiomyopathy (or dysplasia)

Inheritance and genes?

A

Cardiac myocytes replaced by fibrofatty tissue which cannot conduct

AD - desmosomal proteins
AR - non-desmosomal proteins

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42
Q

ECG features of ARVC?

A

QRS prolongation

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43
Q

Treatment of ARVC?

A

avoid competitive sports
B-blockers
ICD if severe

44
Q

What is a mendelian disease?

A

Disease caused by change to a single gene (low prevalence, high penetrance)

45
Q

What test is good for checking more than one gene?

A

Next Gen Sequencing

46
Q

What is the exome?

A

All the parts of the genome that contain genetic information (exons)

47
Q

What amino acid is always the first produced by mRNA?

Codon?

A

Methionine

AUG codon

48
Q

What is cDNA?

A

mature mRNA with the introns spliced out

49
Q

What can help you narrow down the cause of a disorder if they have multiple potential mutations?

A

Phenotype

i.e. no point looking at a mutation for LQTS if they have cardiomyopathy

50
Q

Inheritance of aortic dissection?

A

AD

51
Q

Condition caused by mutation in fibrillar 1 gene?

A

Mafrans

52
Q

Condition similar to Ehlers-Danlos but with aortic aneurysm and dissection?
Inheritance?
Gene?

A

Loeys-Dietz Syndrome

TBR1 or TBR2

AD

53
Q

Familial hypercholesterolaemia:

  • High levels of what?
  • Gene?
  • Inheritance?
A

LDL

LDLR

AD

54
Q

LQTS Romano Ward most common genes?

A

KCNQ1 (LQTS1)

KCNH2 (LQTS2)

55
Q

LQTS Jervell-Lange-Nielsen gene?

A

KCNQ1 (JLN1)

with hearing loss

56
Q

2 most common genes for dissecting aortic aneurysm?

A

ACTA2

Transforming growth factor beta receptor 2

57
Q

What does the duration of clinical death depend on?

A

Length of time the cerebra cortex survives in the absence of circulation and respiration

58
Q

When would you do CPR if someone was still breathing?

A

Agonal breathing

gasping, laboured

59
Q

When should you give IV treatment in CPR?

A

After 3rd round
OR
After delivery of 3rd shock

60
Q

What IV treatment to give in cardiac arrest?

A

IV Adrenaline 1mg

IV Amiodarone 300mg

61
Q

If giving first round of Adrenaline doesn’t work, what is the next step?

A

Continue CPR, give more IV adrenaline every 3-5 mins

62
Q

What shockable condition should you NOT give Amiodarone?

A

TdP

63
Q

Who gets 1st degree heart block?

Description of it?

A

Athletes or those on medications

Regularly irregular

64
Q

MI in which area tends to progress to heart block?

A

Inferior (Right coronary artery)

65
Q

How many polymorphisms do you have?

A

3 million

66
Q

What genetic sequence variant is most likely to cause LQTS

A

Premature stop codon

67
Q

Main piece of advice to give to parents to reduce risk of sudden infant death?

A

Sleep on back, no pillow/toys in cot etc

68
Q

Treatment of LQTS

A

B blocket e.g. atenolol

69
Q

2 reasons why AD mutations may present in only 1 member of the family?

A

De-novo

Mutation is low penetrance, therefore some family members have genotype but no expressed phenotype

70
Q

If you know the mutation and want to test a family member, what genetic test should be done?

A

PCR - don’t need to test whole genome

71
Q

Treatment of familial hypercholesterolaemia?

A

Statin

72
Q

How to differentiate Brugada from MI on ECG?

A

Bruzada has a wide QRS complex, MI doesn’t

73
Q

Drugs to avoid in Brugada syndrome?

A

Sodium channel blockers

Alpha agonists

CCB’s

74
Q

Diagnostic test for Brugada syndrome?

A

Ajmaline testing using ECG

75
Q

Immediate treatment of LQTS?

A

Stop QT prolonging drugs

Correct hypomagnesaemia and hypokalaemia

Initiate a B blocker

Eat lots of K+

76
Q

Ix for suspected TIA from AF?

A

1 - ECHO

2 - Carotid doppler

77
Q

Treatment of carotid plaque-induced stroke/TIA?

A

Anti-platelet

78
Q

Treatment of thrombus-induced stroke/TIA?

A

anticoagulant e.g. warfarin or rivaroxaban

79
Q

Red/white thrombus from AF and carotid plaque?

A

Carotid plaque - platelet rich WHITE thrombus

AF - fibrin rich RED thrombus

80
Q

5 Types of shock?

A 
Anaphylactic 
Cardiogenic
Septic
Hypovolaemic
Neurogenic
81
Q

What is distributive shock?

A

Umbrella term for septic, anaphylactic and neurogenic - Sufficient fluid but in the wrong places

82
Q

What should you give to restore coagulation factory?

A

Calcium

FFP

83
Q

CO = _ x _

A

HR x SV

84
Q

MABP = _ x _

A

CO x TPR

85
Q

Oxygen delivery equation?

A

CO x CaO2 (arterial oxygen content)

86
Q

CO is increased in what type of shock?

A

Septic

87
Q

How can O2 delivery to tissues be improved in shock?

A

Give a blood transfusion

88
Q

BP and HR in anaphylactic shock?

A

BP reduced profoundly

Tachycardia

89
Q

Main pathological process in anaphylactic shock?

A

Mast cell degranulation causes release of inflammatory mediators

90
Q

What type of shock causes bradycardia?

A

Neurogenic

91
Q

What causes bronchoconstriction in anaphylaxis?

A

Leukotrienes

Prostaglandins

92
Q

Treatment of anaphylactic shock? (4)

A
  1. IM Adrenaline
  2. IV fluids
  3. Chlorphenamine
  4. Hydrocortisone
93
Q

BP and HR in cariogenic shock?

A

Low BP

Tachycardic

94
Q

What happens to vessels in Neurogenic shock?

A

vasodilator below lesion

skin may be red and hot

95
Q

Which type of shock causes pulmonary oedema and distended jugular veins?

A

Cardiogenic

96
Q

Treatment of cariogenic shock?

A

Careful fluid management

Inotropes e.g. Dobutamine

Vasopressors e.g. Adrenaline

97
Q

Sepsis 6?

A

BUFALO

B - take bloods
U - measure urine output
F - give fluids
A - give antibiotics
L - measure lactate
O - give oxygen
98
Q

Treatment of neurogenic shock?

A

Vasopressors e.g. adrenaline

99
Q

Septic shock tends to be caused by which type of bacteria?

A

Gram +ve

100
Q

Most common bacteria to cause sudden death in young adults?

A

group A beta haemolytic strep e.g. strep pyogenes

101
Q

Cause of leaky capillaries therefore decreased BP in septic shock?

A

Dysfunctional endothelial barrier as a result of bacteria adhering to it

102
Q

Cause of neurogenic shock?

A

Loss of sympathetic outflow in thoracic spine leading to loss of feedback

103
Q

3 features of qSOFA?

A

Systolic BP <100mmHg

Altered mental status

RR>22

104
Q

Why is HR low in neurogenic shock?

A

Sympathetic outflow taken out by injury so parasympathetic take over by slowing heart down and dilating blood vessels

105
Q

2 shockable rhythms?

A

VF

Pulseless VT

106
Q

4H’s and 4T’s of reversible causes of shock?

A

Hypoxia
Hypovolaemia
Hypothermia
Hyperkalaemia

Tamponade
Tension pneumo
Thrombus
Toxin

Year 3 (19 decks)

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