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YEAR 3 SUDDEN DEATH > Cardiac disease and sudden death > Flashcards

Cardiac disease and sudden death Flashcards

1
Q

what is the prevalence of LQTS

A

1 in 2000

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2
Q

what is the prevalence of HCM

A

1 in 500

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3
Q

what is the prevalence of familial hypercholesterolaemia

A

1 in 500

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4
Q

familial hypercholesterolaemia has mutations in what

A

LDLR
APOB
PCSK9

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5
Q

what is the best genetic test for multifactorial heart disease

A

blood cholesterol

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6
Q

what is sudden cardiac death

A

non-traumatic, non-violent, unexpected death that results from sudden cardiac arrest within 6 hours of previously witnessed normal health

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7
Q

bilateral diagonal earlobe creases and arcus senilis are signs of what

A

hyperlipidaemia

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8
Q

how does fat appear under the microscope

A

clear

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9
Q

blue bag when chest is open implies what

A

pericardium filled with blood

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10
Q

channelopathies are related to an imbalance in what

A

ion current

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11
Q

what is LQTS

A

inherited cardiac condition associated with delayed repolarisation of the ventricles

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12
Q

what can LQTS lead to

A

polymorphic VT (torsades de pointes)

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13
Q

what causes LQTS to go to TdP

A

adrenergic stimulation

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14
Q

what causes LQTS

A

defects in the alpha subunit of the slow delayed rectifier potassium channel

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15
Q

what is the inheritance of LQTS

A

AD or AR

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16
Q

AD LQTS is associated with _______

name 3 of these types

A

extracardiac features

  • Anderson-Tawil syndrome
  • Timothy syndrome
  • Romano Ward
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17
Q

AR LQTS is associated with ______

name this type

A

deafness

- Jervell and Lange-Neillson syndrome

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18
Q

what score is used to determine likelihood of having it

A

Schwartz score

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19
Q

what risk management strategies can someone with LQTS take

A

avoid prescription of drugs which prolong QT interval
avoid sudden surges of exercise (LQTS1)
avoid loud noise e.g. alarm clock (LQTS2)
avoid becoming unwell

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20
Q

name some drugs that prolong QT and so should be avoided

A
  • erythromycin / clarithromycin
  • amiodarone, sotalol, class 1a antiarrhythmic drugs
  • tricyclic antidepressants, selective serotonin reuptake inhibitors (especially citalopram)
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21
Q

how is LQTS treated

A 
avoid risks 
beta blockers (although sotalol may worsen)
ICD in severe
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22
Q

brugada syndrome is a problem with

A

sodium channels

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23
Q

what is the inheritance of brugada syndrome

A

AD

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24
Q

brugada syndrome is more common in men/women

A

men

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25
Q

what arrhythmias are seen in brugada syndrome

A

AF is common

risk of VF/VT

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26
Q

what gene mutations are assoc with brugada syndrome

A

SCN5A (cardiac sodium channel)

CACN1AC (cardiac calcium channel)

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27
Q

brugada syndrome ECG changes may only be seen with provocative testing with

A

flecainide or ajmaline

28
Q

what are some triggers for VF in brugada syndrome

A

rest or sleep
fever
excess alcohol or large meals

29
Q

the genotype and family history doesnt influence prognosis. what does this mean

A

family member dying of it doesnt make it more likely than another family member will die from it

30
Q

what are some risk management strategies with brugada syndrome

A

avoid drugs that might increase ST elevation
early paracetamol in fever
avoid excess alcohol or food

31
Q

what is the management of brugada syndrome

A

ICD

32
Q

what is a condition that is very rare with a very high risk of arrhythmia and people with it usually die in early childhood

A

short QT syndrome

33
Q

short QT syndrome is due to mutation in what channels

A

K+

34
Q

what is CPVT

A

catecholaminergic polymorphic ventricular tachycardia

- adrenergic induced bidirectional and polymorphic VTs and SVTs

35
Q

what is the inheritance of CPVT

A

AD

36
Q

CPVT is a problem with what

A

ranitidine receptor

37
Q

what arrhythmias are seen in CPVT

A

polymorphic VT

SVTs

38
Q

what is the prevalence of CPVT

A

1 in 10,000

39
Q

what would an ECG/echo of CPVT look like

A

normal

40
Q

what is the treatment of CPVT

A

high dose BB and ICD

sometimes treat with flecainide

41
Q

what triggers the arrhythmias in CPVT

A

emotional stress, physical activity

42
Q

people with CPVT should avoid

A

competitive sports

43
Q

what causes WPW

A

accessory pathway from atria to ventricles bypassing the AV node leading to atrioventricular re-entry tachycardia (AVRT)

44
Q

what can AF progress to in WPW and why

A

VF - no slowing down of conduction through accessory pathway

45
Q

how is WPW diagnosed

A

on treadmill to see if they can still conduct at maximum HR

46
Q

how does HCM present

A

HF or sudden death

47
Q

when should you consider an ICD in HCM

A

risk of sudden death > 4%

48
Q

people with HCM should avoid

A

competitive sport

49
Q

where does the problem lie with HCM

A

output difficulty due to huge mass of muscle

50
Q

the mutation is in what genes in HCM

A

sarcomeric

51
Q

DCM is more common in males/females

A

males

52
Q

what is the inheritance of HCM

A

AD

53
Q

what is the inheritance of DCM

A

AD

54
Q

what happens in DCM

A

slowly over time the heart muscles dilate and scar leading to rhythm problem

55
Q

what is ARVC

A

arrhythmogenic right ventricular cardiomyopathy

- fibro-fatty replacement of cardio myocytes

56
Q

what is the inheritance of ARVC

A

AD but can be AR

57
Q

is ARVC easy or difficult to treat

A

difficult

58
Q

how is ARVC treated

A

ICD and BB (sotalol)

59
Q

people with ARVC should avoid

A

competitive sports

60
Q

what kind of screening is important with inherited cardiac conditions

A

familial (cascade) screening

61
Q

where do the 2 leads of an ICD go

A

one to right ventricle

one to right atrium

62
Q

what is the risk of death when taking out leads of an ICD

A

1 in 100

63
Q

what are some complications of an ICD

A

endocarditis, perforation, haemothorax, pneumothorax, embolism, leads can break or move or deliver shock when not required

64
Q

what is an after depolarisation

A

abnormal depolarisation of cardiac myocytes

65
Q

EADs cause

A

TdP

66
Q

what is automaticity

A

SA node fires generating a heart beat without any external stimulus

YEAR 3 SUDDEN DEATH (7 decks)

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