Cardiac Arrythmias Flashcards

1
Q

Who’s consent do you require to do a hospital autopsy?

A

next of kin or family

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2
Q

What are the circumstances that a procurator fiscal would instruct an autopsy?

A
sudden and unexplained death
suspected suicide or homocide
death of a child
death at work 
death due to medical or dental care
death in custody
death due to an industrial or notifiable disease
death due to an accident
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3
Q

What are the two broad catagories of inherited cardiac arrythmias?

A

cardiomyopaties

channelopathies

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4
Q

What causes channelopathies?

A

ion imbalance and development of early and late depolarisations

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5
Q

Give some examples of a channelopathy?

A
long QT syndrome
brugada syndrome
catecholaminergic polymorphic ventricular tachycardia (CPVT)
short QT syndrome
familial AF
WPW
progressive familial conduction disease
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6
Q

What causes cardiomyopathies?

A

scar or electrical barrier formation causing re entry

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7
Q

What are examples of cardiomyopathies?

A

hypertrophic cardiomyopathy
arrythmogenic right ventricular cardiomyopathy (ARVC)
dilated cardiomyopathy

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8
Q

What is an after depolarisation?

A

abnormal depolarisation of cardiac myocytes that interrups phase 2,3 or 4 of the cardiac AP

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9
Q

What are early after depolarisations?

A

abnormal depolarisation in phase 2 or 3

caused by increased frequency of abortive action potentials before normal repolarisation is completed

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10
Q

What can early after depolarisations result in?

A

torsades de pointes

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11
Q

What can make early after depolarisations worse?

A

hypokalaemia

drugs that prolong the QT interval

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12
Q

What are delayed after depolarisations?

A

begin during phase 4 - after repolarisation is complete but before another action potential would normally occur

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13
Q

What causes delayed after depolarisations?

A

elevated Ca
seen in digoxin toxicity
overload of the SR can cause spontaneous Ca release

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14
Q

What is the classical feature of delayed after depolarisations?

A

bidirectional ventricular tachycardia

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15
Q

In what condition do delayed after depolarisations occur?

A

CPVT

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16
Q

What is the incidence of congenital long QT syndrome?

A

1 in 2000 are carriers

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17
Q

What is long QT syndrome?

A

polymorphic VT (i.e. torsades de pointes) due to adrenergic stimulation

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18
Q

What are the autosomal dominant varieties of long QT?

A

isolated: Romano Ward Syndrome

extra cardiac features: Anderson-Twail syndrome, Timothy syndrome

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19
Q

What are the autosomal recessive varieties of long QT?

A

associated with deafness: Jervell + Lange-Nielsen Syndrome

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20
Q

What causes long QT syndrome?

A

genetic mutations that cause reduced or dysfunctional ionic current - causing prolonged cardiac repolarisation
more depolarising current - Na and Ca
less repolarising current - K

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21
Q

How is LQTS diagnosed?

A

QTc>/= 480ms

22
Q

What factors contribute to sudden death in LQTS?

A

age dependant
gender - pre adolescent males and adult females
increasing QT duration
prior syncope and response to beta blockers

23
Q

How is LQTS managed?

A

lifestyle - avoid stress, strenuous exercise, loud noise if recessive, strenuous swimming if dominant
beta blockers
ICD
avoid QT prolonging drugs

24
Q

What drugs are QT prolonging?

A
antipsychotics - haloperidol
antibiotics - macrolides (erythromycin)
anti arrythmics - class 1a and 3
anti emetics - ondesantron
anti depressants - tricyclics
25
Q

What causes congenital short QT syndrome?

A

mutation in the K channels

26
Q

How is congenital short QT syndrome diagnosed?

A

QT <300ms at a HR of <80bpm

27
Q

How does congenital short QT present?

A

often in young children

associated with AF

28
Q

What is Brugada syndrome?

A

VT and VF due to triggers for eg - sleep, fever, alcohol, large meals

29
Q

What causes Brugada syndrome?

A

genetic mutations for eg SCN5A and CAN1AC

30
Q

How is Brugada syndrome inherited?

A

autosomal dominant

31
Q

Who is at higher risk of Brugada syndrome?

A

females x 8

32
Q

How does Brugada syndrome look on ECG?

A

ST elevation >2cm in V1 +/- V2

RBBB in V1-V3

33
Q

How can you elict Brugada syndrome?

A

flecainide

34
Q

How does flecainide stimulate Brugada syndrome?

A

blocks the cardiac Na channel

35
Q

How do you treat/manage Brugada syndrome?

A

avoid drugs that induce ST elevation
avoid excessive alcohol or large meals
ICD for those with previous cardiac arrest or type 1 ECG pattern or history of syncope

36
Q

What drugs induce ST elevation?

A

anti arrythmics
psychotropics
analgesics
anaesthetics

37
Q

What is Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)?

A

adrenergic induced bidirectional and polymorphic VT, SVT triggered by emotional stress and activity

38
Q

How does CPVT appear on ECG and ECHO?

A

normal

39
Q

How is CPVT inherited?

A

autosomal dominant or recessive

40
Q

How can CPVT be managed?

A

lifestyle - avoid emotional and physical stress
beta blockers
ICD - if contraindicated give flecanide

41
Q

How does Wolff-Parkinson-White syndrome present?

A

AVRT and AF

small risk of sudden death

42
Q

How does Wolff-Parkinson-White look on ECG?

A

delta wave and short PR interval due to ventricular pre excitation due to the accessory pathway

43
Q

How is hypertrophic cardiomyopathy inherited?

A

autosomal dominant

44
Q

What gene is mutated in hypertrophic cardiomyopathy?

A

sarcomere gene

45
Q

How does hypertrophic cardiomyopathy present?

A

AF
heart failure
sudden death

46
Q

How is hypertrophic cardiomyopathy treated?

A

lifestyle - avoid emotional and physical stress

ICD if prior cardiac arrest/sustained VT or if LV wall thickness >/= 30mm

47
Q

What genes are affected in dilated cardiomyopathy?

A

lamnin A/C and desmin

dystrophin if X linked

48
Q

Who is more affected by dilated cardiomyopathy - males or females?

A

males

49
Q

What is Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)?

A

fibrofatty replacements of cardiomyocytes

LV involvement in >50% of cases

50
Q

How is ARVC passed on?

A

autosomal dominant - genes for desmosomal proteins

autosomal recessive - genes for non desmosomal proteins

51
Q

What does ARVC cause?

A

a reentry VT

52
Q

How is ARVC managed?

A

avoid competitive sport
beta blockers
ICD