Sudden Death Flashcards by Joe McAuley

3 inherited cardiac causes of sudden death?

Inherited arrhythmia syndrome

Inherited cardiomyopathy

Inherited multi system disease with CVS involvement, e.g. myotonic dystrophy

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Another name for arrhythmia syndromes?

Channelopathies

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6 inherited channelopathies

Congenital LQTS

Brugada syndrome

Catecholaminergic polymorphic VT

Short QT syndrome

Familial AF

WPW

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An inherited cardiomyopathy

hypertrophic cardiomyopathy

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Screening is routinely offered for which type of cardiomyopathy?

Dilated

Hypertrophic

Arrhythmogenic right ventricular cardiomyopathy

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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

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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

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Inheritance pattern of cardiac conditions?

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Most common inherited cardiac cause of sudden death?

How many people are carriers for the gene?

LQTS

1 in 2000

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What arrhythmia is caused by LQTS?

What triggers the arrhythmia’s?

Torsades de Pointes
(polymorphic VT)

Triggered by adrenergic stimulation, e.g. excitement, exercise

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What is Romano Ward Syndrome?

Inheritance?

Most common form of LQTS

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What LQTS is assoc w deafness?

Inheritance?

Jervell and Lange-Nielsen Syndrome

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Pathophysiology behind LQTS?

Reduced ionic current = prolonged repolarisation = QT interval prolongation

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How is LQTS diagnosed?

QT>480ms (>0.48s/>2 big boxes and 2 small boxes)

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Symptoms of LQTS?

Syncope
Stress
Potentially HX of deafness (JALNS)

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5 meds that should be avoided in LQTS?

Clarithromycin
Azithromycin
Imipramine
Amitriptyline
Haloperidol

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LQTS1 prophylactic advice?

Avoid strenuous exercise

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LQTS2 prophylactic advice?

Avoid loud noises

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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

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SQTS puts you at risk of what arrhythmia?

TdP (polymorphic VT)

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What arrhythmia do people with Brugada syndrome get?

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ECG abnormalities in Brugada syndrome?

ST elevation and RBBB affecting V1-V3

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Inheritance of Brugada syndrome?

Who gets it?

Young males

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Triggers for VF in Brugada Syndrome?

Usually sleep or rest
Fever
Excess alcohol
Large meals

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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?

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?

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)

1. IM Adrenaline 2. IV fluids 2. Chlorphenamine 4. 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

Sudden Death Flashcards

(106 cards)