Cardiac Causes of Sudden Death

Question 1

What are the three broad categories of inherited cardiac conditions that may present as sudden death?

Answer 1

inherited arrhythmia syndromes
Inherited cardiomyopathy
Inherited multisystem diseases with CVS involvement (e.g. myotonic dystrophy)

Question 2

what would ECG screening pick up?

Answer 2

channelopathies

cardiomyopathies

Question 3

triggered activity of the heart may be caused by…

Answer 3

after deoplarizations

Question 4

a longer QT interval means a higher chance of what?

Answer 4

an extra depolarization

Question 5

Syndrome caused by a sodium channel problem?

Answer 5

Brugada syndrome

Question 6

which syndrome is very rare and people with it usually die in childhood?

Answer 6

Short QT syndrome

Question 7

how many people are carriers of a long QT gene?

Answer 7

1/2000

Question 8

what can trigger polymorphic VT (Torsades de pointes)?

Answer 8

adrenergic stimulation

Question 9

what is the risk of sudden death in untreated long QT syndrome?

Answer 9

under 1%

Question 10

What proportion of people with long QT experience syncope episodes annually?

Answer 10

5%

Question 11

how high would your risk of cardiac arrest from long QT syndrome need to be to have an ICD?

Answer 11

4%

Question 12

what scoring system is used to calculate likelihood of LQTS?

Answer 12

schwartz score

Question 13

what are some lifestyle changes to make to prevent an event with LQTS?

Answer 13

avoid OT prolonging drugs
correct electrolyte abnormalities (e.g. when have diarrhoea, vomiting etc.)
avoid genotype specific features- some triggered by strenuous exercise/ loud noises e.g. alarm clocks)

Question 14

What does Brugada syndrome put you at risk of?

Answer 14

polymorphic VT or VF

Question 15

what does Brugada syndrome look like on ECG?

Answer 15

ST elevation in RBBB in V1,2,3

Question 16

females are 8X more likely to get brugada syndrome TRUE/ FALSE

Answer 16

FALSE

males

Question 17

what triggers Brugada syndrome?

Answer 17

rest/sleeping
fever
ST elevating drugs
excessive alcohol/food

Question 18

what is the incidence of arrhythmia from Brugada syndrome in asymptomatic people?

Answer 18

1%

Question 19

what is the incidence of arrhythmia from Brugada syndrome in patients with syncope?

Answer 19

3.2%

Question 20

what is the incidence of arrhythmia from Brugada syndrome in patients who have previously had a cardiac arrest?

Answer 20

13.5%

Question 21

If a person with Briugada syndrome is coming down with a fever, what must they do?

Answer 21

take early paracetamol!!!

Question 22

What is CPVT?

Answer 22

chatecholaminergic polymorphic ventricular tachycardia

Question 23

describe the pathophysiology of CPVT

Answer 23

abnormal response to adrenaline.

problem in RYANODINE receptor

Question 24

what arrhythmia can be a result of CPVT?

Answer 24

Bi-directional VT

Question 25

Name two treatments for CPVT?

Answer 25

beta blockers- v tight control

ICD

Question 26

what is the most common arrhythmia with Wolff-Parkinson White Syndrome?

Answer 26

Atrioventricular re-entrant tachycardia

Question 27

how does WPW show on ECG?

Answer 27

short PR interval and delta wave

Question 28

where is the mutation in 60% of hypertrophic cardiomopathies?

Answer 28

sarcomere genes

Question 29

what is a big trigger of sudden death from hypertrophic cardiomyopathy?

Answer 29

strenuous exercise

Question 30

dilated cardiomyopathies are more common in males, true/false?

Answer 30

TRUE

Question 31

which cardiomyopathy involves fibro-fatty replacement of cardio myocytes?

Answer 31

arrhythmogenic right ventricular cardiomyopathy

Question 32

what mode of inheritance is ARVC usually?

Answer 32

autosomal dominant