cardiac conditions that cause sudden death Flashcards

1
Q

what is considered a long QT interval usually

A

>450ms

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2
Q

what happens in long QT syndrome

A

the ventricular repolarizationis prolonged - long QT interval

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3
Q

romano ward syndrome

A

autosomal dominant cuase of long QT syndrome - causes isolated long QT

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4
Q

JLN syndrome

A

autosomal recessive cause of congenital long QT - associated with congenital deafness

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5
Q

why does hypokalaemia cause acquired long QT

A

K leaving the cell si responsible for stages 2 3 and 4 of the AP

if there is less K, prolongs the plateau phase

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6
Q

what 2 other electrolyte abnormalities cause acquried long QT

A

hypoMg

hypoCa

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7
Q

which ABx cause long QT

A

macrolides eg azithromycin

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8
Q

what other drugs cause long QT

A

antidepressants, TCA and citalopram

antipsychotics

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9
Q

which class of anti arrhythmic drugs prolonged the plateau phase of the cardiac AP

A

3 - amiodarone and sotalol

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10
Q

what do the LGT genes encode

A

cardiac potassium and sodium channels

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11
Q

what is the genetics cause of long qt

A

heterogenous - caused by lots of different genes

each different gene causes a disease with a different phenotype

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12
Q

what are the particular provocations for LGT 1 2 and 3

A

1 - strenuous exericse, especially swimming

2 - emotion and loud noises

3 - rest or sleep

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13
Q

what can long at lead to and what are the symptoms

A

torsade de pointes - a polymorphic ventricular tachycardia

syncope and palpitations

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14
Q

what is the danger of TdP

A

it can degenerate into ventricular fibrillation

can terminate spontaneously

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15
Q

what is assoicated with increased risk in long qt syndrome

A

the length of the qt interval

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16
Q

what treatment is advised for congenital long qt syndrome

A
  • avoid triggers
  • beta blockers (not sotalol)
  • permanent pacing
  • ICD
17
Q

who is eligible for an ICD with congenital long qt

A

those resuscitated from cardiac arrest or those with syncope despite beta blocker treatment

18
Q

genes encoding which channels are mutated in short QT syndrome

A

K channels

19
Q

short QT syndrome presentation

A

usually seen in young children adn they often dont survive, it is a cause of SIDS

assoicated with AF, ventricular arhythmias and suden death

20
Q

management of short QT syndrome

21
Q

what is the classical ECG of brugada syndrome

A

ST elevation, inverted T wave, RBBB in V1-3

22
Q

does someone with brugada syndrome have ecg changes all the time?

A

no, may be intermittent and change over time

often seen with fever

23
Q

what drugs can be used to provoke ecg changes in brugada syndrome

A

class 1 anti arrhythmics - flecainide or ajmoline

24
Q

what are some triggers for VF in brugada syndrome

A

rest, sleep, fever, aalcohol, large meals

25
what drug should be avoided in brugada syndrome
beta blockers
26
what is the management of brugada syndrome
ICD - advised in those who have a cardiac arrest, abnormal ecg or collapse
27
what is the inheritance of CPVT
au dominant
28
what happens in CPVT
catecholamine release in response to activity or stress causes sympathetic stimulation of the heart - leads to an increase in IC calcium ion concentration and results in an arrhythmia cascade
29
mutations in which genes are associated with CPVT
RYR2 or CASQ2
30
what arrhythmia does CPVT cause
bidirectional ventricular tachycardia
31
how does CPVT present
with syncope or sudden death on activity/stress
32
what are the investigations of CPVT like when it is not being provoked
normal at baseline
33
why is management of CPVT hard
a cardiac event or defibrillation cause catecholamine release which trigger the arrhythmia. so does hospital environemnet and stress
34
management of CPVT
beta blockers fleicainide ICD
35
what happens in WPW syndrome
there is a congneital anatomical re entrant circuit between the atria and ventricles called the bundle of Kent - this doesnt have a refractory period so allows electrical signals to go back from ventricles to atria really fast
36
what is the risk in WPW linked to
how fast the bundle of kent can transmit signals
37
ecg changes in WPW
delta wave on ecg - slurred upstroke on QRS complex short PR interval