Sudden death

Question 1

sudden death definition;

age
timing

Answer 1

<50yo

<6 hours of being previously well

Question 2

inheritance pattern of most cardiac inherited conditions

Answer 2

autosomal dominant

Question 3

what is the most common inherited cardiomyopathy

Answer 3

hypertrophic cardiomyopathy

Question 4

aetiology of sudden death (3)

Answer 4

shock
sepsis
cardiac causes - inherited

Question 5

most common inherited arrhythmia syndrome that causes sudden death

Answer 5

long QT syndrome

Question 6

inherited arrhythmias that cause sudden death (5)

Answer 6

long QT 
brugada 
short QT 
catecholinergic polymorphic VT 
wolff Parkinson white syndrome

Question 7

inherited cardiomyopathies that cause sudden death (3)

Answer 7

hypertrophic cardiomyopathy
arrythmogenic right ventricular cardiomyopathy
dilated cardiomyopathy

Question 8

which MSK condition is abdominal aortic aneurysms connected to

why

Answer 8

marfans

fibrillin mutation = connective tissue disorder

Question 9

example of QT prolonging drug (3)

Answer 9

azithromycin, clarithromycin

second generation atypical antipsychotics eg olanzapine, quetiapine, risperidone

SSRIs - citalopram

Question 10

what syndrome is caused by autosomal dominant inheritance of long QT

do any other symptoms present?

Answer 10

ramano-ward syndrome

no just QT

(THINK 1 part to syndrome name = 1 symptom)

Question 11

what syndrome is caused by autosomal recessive inheritance of long QT

do any other symptoms present?

Answer 11

jervell + lange-nielsen syndrome

deafness + long QT

(THINK 2 parts to syndrome name = 2 symptoms)

Question 12

ECG of long QT syndrome (2)

Answer 12

QT >480ms (T wave finishes over half way between S and Q) 
polymorphic VT (torsades de pointes)

Question 13

fourth rail bridge on ECG

Answer 13

torsades de pointes = long QT syndrome

Question 14

what is the QRS like in polymorphic VT

Answer 14

broad QRS (+ ventricular problem)

Question 15

are there p waves in polymorphic VT

Answer 15

no p waves

Question 16

what is the equation for calculating QT length depending on heart rate

Answer 16

QT length divided by square root of R-R interval

Question 17

treatment of long QT syndrome

Answer 17

beta blockers

Question 18

inheritance pattern of brugada syndrome

Answer 18

autosomal dominant

Question 19

ECG findings for brugada syndrome (2)

Answer 19

‘hash brown’/coved shaped ST elevation in V1-V3

RBBB

Question 20

what does RBBB look like

Answer 20

M in V1
W in V6

MaRRoW

Question 21

which group of people does short QT present in

is it rare or common

prognosis

Answer 21

children

rare

kills them!

Question 22

triggers of arrhythmias

Answer 22

intense exercise/stress (Adrenaline)

Question 23

delta wave on ECG

Answer 23

wolff Parkinson white syndrome

Question 24

acute treatment of wolff Parkinson white syndrome

Answer 24

ablation

Question 25

which protein is altered in hypertrophic cardiomyopathy

Answer 25

myosin binding protein C

Question 26

which ventricle is thickened in hypertrophic cardiomyopathy

Answer 26

left ventricle hypertrophy

Question 27

young athlete collapsed on pitch | sudden death caused by heart failure

Answer 27

hypertrophic cardiomyopathy

Question 28

ECG findings in hypertrophic cardiomyopathy

Answer 28

tall QRS (>7 boxes in V2 and V5)

Question 29

tall QRS

Answer 29

ventricular hypertrophy

Question 30

what should you do to the families of anyone with an inherited cardiomyopathy/arrhythmia

Answer 30

family screening

Question 31

if hypertrophic cardiomyopathy, what should they avoid

Answer 31

intense exercise

Question 32

autosomal dominant inheritance in most congenital cardiac conditions, what is the chance of child being affected

Answer 32

1 in 2

Question 33

which gene causes half of arrythmogenic right ventricular cardiomyopathy

Answer 33

PKP2 gene

Question 34

what does having PKP2 gene mean in terms of penetrance and expression

Answer 34

low penetrance - not everyone with gene will have disease highly variable expression - a range of presentations, some asymptomatic, some will die in relation to arrythmogenic right ventricular cardiomyopathy

Question 35

ECG in arrythmogenic right ventricular cardiomyopathy

Answer 35

broad QRS (= ventricular problem)

Question 36

if you had to guess in exams, how would you treat an inherited cardiac problem

Answer 36

beta blocker | avoid exercise/competitive sports

Question 37

what do you need to do before you confirm death in a neonate

Answer 37

pat nappy

Question 38

what do you need to do after confirmation of sudden/unexpected neonatal death

Answer 38

procurator fiscal

Question 39

what can happen to the other children of people who have had neonatal bereavement

Answer 39

neglect

Question 40

methods to prevent cot death

Answer 40

clear cot lie on back feet at bottom - so they cant wriggle down under covers

Question 41

what do doctors do to verify death

Answer 41

``` pulse - 1-2min, 2 pulses pupil reflexes - with torch, lens clouding auscultate - breathing and heart 1 min observe movement and breathing reaction to voice or pain (GCS = 3) NOTE if there's a pacemaker ```

Question 42

who orders a post mortem

Answer 42

procurator fiscal