Sudden Death Flashcards
3 inherited cardiac causes of sudden death?
Inherited arrhythmia syndrome
Inherited cardiomyopathy
Inherited multi system disease with CVS involvement, e.g. myotonic dystrophy
Another name for arrhythmia syndromes?
Channelopathies
6 inherited channelopathies
Congenital LQTS
Brugada syndrome
Catecholaminergic polymorphic VT
Short QT syndrome
Familial AF
WPW
An inherited cardiomyopathy
hypertrophic cardiomyopathy
Screening is routinely offered for which type of cardiomyopathy?
Dilated
Hypertrophic
Arrhythmogenic right ventricular cardiomyopathy
When do early depolarisations occur?
What arrhythmia do they cause?
What Potentiates them?
Phases 2 and 3 of cardiac AP
Torsades de Pointes
Hypokalaemia and Class Ia and III anti-arrhythmatics which prolong QT interval
When do delayed depolarisations occur?
What inherited condition are they seen in?
What arrhythmia do they cause?
phase 4 of cardiac AP
Catecholaminergic polymorphic ventricular tachycardia
Bidirectional VT
Inheritance pattern of cardiac conditions?
AD
Most common inherited cardiac cause of sudden death?
How many people are carriers for the gene?
LQTS
1 in 2000
What arrhythmia is caused by LQTS?
What triggers the arrhythmia’s?
Torsades de Pointes
(polymorphic VT)
Triggered by adrenergic stimulation, e.g. excitement, exercise
What is Romano Ward Syndrome?
Inheritance?
Most common form of LQTS
AD
What LQTS is assoc w deafness?
Inheritance?
Jervell and Lange-Nielsen Syndrome
AR
Pathophysiology behind LQTS?
Reduced ionic current = prolonged repolarisation = QT interval prolongation
How is LQTS diagnosed?
QT>480ms (>0.48s/>2 big boxes and 2 small boxes)
Symptoms of LQTS?
Syncope
Stress
Potentially HX of deafness (JALNS)
5 meds that should be avoided in LQTS?
Clarithromycin Azithromycin Imipramine Amitriptyline Haloperidol
LQTS1 prophylactic advice?
Avoid strenuous exercise
LQTS2 prophylactic advice?
Avoid loud noises
What is affected in SQTS (short QT)?
How long does it have to be for this?
Who’s is affected by SQTS?
K+ channels
<300ms (less than 1 big box and 3 small boxes) at HR <80bpm
Young children
SQTS puts you at risk of what arrhythmia?
TdP (polymorphic VT)
VF
What arrhythmia do people with Brugada syndrome get?
AF
ECG abnormalities in Brugada syndrome?
ST elevation and RBBB affecting V1-V3
Inheritance of Brugada syndrome?
Who gets it?
AD
Young males
Triggers for VF in Brugada Syndrome?
Usually sleep or rest
Fever
Excess alcohol
Large meals
Channels and genes assoc w Brugada syndrome?
Cardiac sodium channel (SNC5A)
Calcium channel (CACN1Ac)
Treatment of Brugada Syndrome?
Avoid triggers
Avoid Na channel blocker/anti-arrhythmatics
Go into hospital if fever
ICD if severe
What is catecholaminergic polymorphic ventricular tachycardia?
Triggers?
adrenergic-induced bidirectional and polymorphic VT and SVT’s
Stress (e.g. alarm clocks, jumping in cold pool), physical activity
ECG in CPVT?
usually normal unless induced arrhythmia, echo also normal
Autosomal dominant CPVT gene?
Autosomal recessive CPVT gene?
Dominant - Ryanodine receptor mutation (RyR2)
Recessive - Cardiac Calsequestrin gene (CASQ2)
Treatment of CPVT?
Prophylaxis?
Defibrillate if necessary
Prophylaxis:
- avoid triggers
- B-blockers
- +/- Flecainide
- +/- ICD
3 ECG features of WPW?
Short PR
Delta waves
Ventricular pre-excitation
Most common arrhythmias in WPW?
AV re-entry tachycardia
AF
Pathophysiology of WPW?
No conduction delay between atria and ventricles due to an accessory pathway bypassing AV node
Treatment for WPW?
Leave it if accessory pathway does not conduct quickly
Otherwise, ablate the pathway
Most common cardiomyopathy?
What type of mutation is most common? What gene is affected most commonly?
Hypertrophic
Sarcomere gene mutation - most common MYBPC3
What % of patients with HOCM die each year?
1%
2 ECG changes that suggest hypertrophy?
Positive deflection in V5
Negative deflection in V2
Treatment of HOCM?
ICD if arrest, sustained VT or high risk e.g. 1st degree FHx, thick wall, recent syncope
If none then leave it as risk of cardiac event <4%
Who gets dilated cardiomyopathy?
adulthood-elderly
Men
Genes and structures related to dilated cardiomyopathy?
Sarcomere and desmosomal genes
Lamina A/C if there is conduction disease
Dystrophin gene if X-linked (muscular dystrophy)
Pathophysiology of arrhythmogenic right ventricular cardiomyopathy (or dysplasia)
Inheritance and genes?
Cardiac myocytes replaced by fibrofatty tissue which cannot conduct
AD - desmosomal proteins
AR - non-desmosomal proteins
ECG features of ARVC?
QRS prolongation
Treatment of ARVC?
avoid competitive sports
B-blockers
ICD if severe
What is a mendelian disease?
Disease caused by change to a single gene (low prevalence, high penetrance)
What test is good for checking more than one gene?
Next Gen Sequencing
What is the exome?
All the parts of the genome that contain genetic information (exons)
What amino acid is always the first produced by mRNA?
Codon?
Methionine
AUG codon
What is cDNA?
mature mRNA with the introns spliced out
What can help you narrow down the cause of a disorder if they have multiple potential mutations?
Phenotype
i.e. no point looking at a mutation for LQTS if they have cardiomyopathy
Inheritance of aortic dissection?
AD
Condition caused by mutation in fibrillar 1 gene?
Mafrans
Condition similar to Ehlers-Danlos but with aortic aneurysm and dissection?
Inheritance?
Gene?
Loeys-Dietz Syndrome
TBR1 or TBR2
AD
Familial hypercholesterolaemia:
- High levels of what?
- Gene?
- Inheritance?
LDL
LDLR
AD
LQTS Romano Ward most common genes?
KCNQ1 (LQTS1)
KCNH2 (LQTS2)
LQTS Jervell-Lange-Nielsen gene?
KCNQ1 (JLN1)
with hearing loss
2 most common genes for dissecting aortic aneurysm?
ACTA2
Transforming growth factor beta receptor 2
What does the duration of clinical death depend on?
Length of time the cerebra cortex survives in the absence of circulation and respiration
When would you do CPR if someone was still breathing?
Agonal breathing
gasping, laboured
When should you give IV treatment in CPR?
After 3rd round
OR
After delivery of 3rd shock
What IV treatment to give in cardiac arrest?
IV Adrenaline 1mg
IV Amiodarone 300mg
If giving first round of Adrenaline doesn’t work, what is the next step?
Continue CPR, give more IV adrenaline every 3-5 mins
What shockable condition should you NOT give Amiodarone?
TdP
Who gets 1st degree heart block?
Description of it?
Athletes or those on medications
Regularly irregular
MI in which area tends to progress to heart block?
Inferior (Right coronary artery)
How many polymorphisms do you have?
3 million
What genetic sequence variant is most likely to cause LQTS
Premature stop codon
Main piece of advice to give to parents to reduce risk of sudden infant death?
Sleep on back, no pillow/toys in cot etc
Treatment of LQTS
B blocket e.g. atenolol
2 reasons why AD mutations may present in only 1 member of the family?
De-novo
Mutation is low penetrance, therefore some family members have genotype but no expressed phenotype
If you know the mutation and want to test a family member, what genetic test should be done?
PCR - don’t need to test whole genome
Treatment of familial hypercholesterolaemia?
Statin
How to differentiate Brugada from MI on ECG?
Bruzada has a wide QRS complex, MI doesn’t
Drugs to avoid in Brugada syndrome?
Sodium channel blockers
Alpha agonists
CCB’s
Diagnostic test for Brugada syndrome?
Ajmaline testing using ECG
Immediate treatment of LQTS?
Stop QT prolonging drugs
Correct hypomagnesaemia and hypokalaemia
Initiate a B blocker
Eat lots of K+
Ix for suspected TIA from AF?
1 - ECHO
2 - Carotid doppler
Treatment of carotid plaque-induced stroke/TIA?
Anti-platelet
Treatment of thrombus-induced stroke/TIA?
anticoagulant e.g. warfarin or rivaroxaban
Red/white thrombus from AF and carotid plaque?
Carotid plaque - platelet rich WHITE thrombus
AF - fibrin rich RED thrombus
5 Types of shock?
Anaphylactic Cardiogenic Septic Hypovolaemic Neurogenic
What is distributive shock?
Umbrella term for septic, anaphylactic and neurogenic - Sufficient fluid but in the wrong places
What should you give to restore coagulation factory?
Calcium
FFP
CO = _ x _
HR x SV
MABP = _ x _
CO x TPR
Oxygen delivery equation?
CO x CaO2 (arterial oxygen content)
CO is increased in what type of shock?
Septic
How can O2 delivery to tissues be improved in shock?
Give a blood transfusion
BP and HR in anaphylactic shock?
BP reduced profoundly
Tachycardia
Main pathological process in anaphylactic shock?
Mast cell degranulation causes release of inflammatory mediators
What type of shock causes bradycardia?
Neurogenic
What causes bronchoconstriction in anaphylaxis?
Leukotrienes
Prostaglandins
Treatment of anaphylactic shock? (4)
- IM Adrenaline
- IV fluids
- Chlorphenamine
- Hydrocortisone
BP and HR in cariogenic shock?
Low BP
Tachycardic
What happens to vessels in Neurogenic shock?
vasodilator below lesion
skin may be red and hot
Which type of shock causes pulmonary oedema and distended jugular veins?
Cardiogenic
Treatment of cariogenic shock?
Careful fluid management
Inotropes e.g. Dobutamine
Vasopressors e.g. Adrenaline
Sepsis 6?
BUFALO
B - take bloods U - measure urine output F - give fluids A - give antibiotics L - measure lactate O - give oxygen
Treatment of neurogenic shock?
Vasopressors e.g. adrenaline
Septic shock tends to be caused by which type of bacteria?
Gram +ve
Most common bacteria to cause sudden death in young adults?
group A beta haemolytic strep e.g. strep pyogenes
Cause of leaky capillaries therefore decreased BP in septic shock?
Dysfunctional endothelial barrier as a result of bacteria adhering to it
Cause of neurogenic shock?
Loss of sympathetic outflow in thoracic spine leading to loss of feedback
3 features of qSOFA?
Systolic BP <100mmHg
Altered mental status
RR>22
Why is HR low in neurogenic shock?
Sympathetic outflow taken out by injury so parasympathetic take over by slowing heart down and dilating blood vessels
2 shockable rhythms?
VF
Pulseless VT
4H’s and 4T’s of reversible causes of shock?
Hypoxia
Hypovolaemia
Hypothermia
Hyperkalaemia
Tamponade
Tension pneumo
Thrombus
Toxin