Cardiac Conditions that Cause Sudden Death Flashcards
Define sudden cardiac death
Event that is non-traumatic, non-violent, unexpected and resulting from sudden cardiac arrest within 6hrs of previously witnessed normal health
Is Brugada syndrome a channelopathy or cardiomyopathy?
Channelopathy
What is a channelopathy?
Ion current imbalance + development of early/late depolarizations
What is a cardiomyopathy?
Arrhythmogenesis related to scar/electrical barrier formation and subsequent re-entry
Are channelopathies normal or abnormal at screening?
Often normal
Are cardiomyopathies normal or abnormal at screening?
Often abnormal
What are the mechanisms of arrhythmia?
Automaticity - thing that keeps node firing
Trigger
Re-entry e.g. scar
When do ‘after depolarizations’ occur?
interrupt phase 2,3 or 4 of cardiac action potential
What do after depolarizations lead to?
Triggered activity - sustained cardiac arrhythmia e.g. fibrillation
When do early after depolarizations occur?
During phase 2 or 3
What happens during phase 4 of the cardiac action potential?
Repolarization phase
Ventricular myocyte at rest
K+ channels open
Resting membrane potential = -90mV
What happens during phase 0 of the cardiac action potential and why?
Fast Na+ influx
Depolarization
What happens during phase 1 of the action potential cardiac cycle?
Closure of Na+ channels and transient K+ efflux
What happens during phase 2 of the action potential cycle?
Ca++ influx
Plateau
What happens during phase 3 of the cardiac action potential cycle?
Closure of Ca++ channels and K+ efflux
Rapid repolarization
What type of activity do after depolarizations lead to?
Triggered activity - sustained cardiac arrhythmia
What causes an early after depolarization?
Increase in the frequencyof abortive action potentials before normal repolarization is complete
Why might phase 2 be interrupted by an early afterdepolarization?
Augmented opening of Ca2+ channels
Why might phase 3 be interrupted by an early afterdepolarization?
Opening of Na2+ channels
What do early afterdepolarizations cause?
Torsades de Pointes
What is Torsades de Pointes?
Specific form of VT in patients with long QT syndrome.
What might Torsades de Pointes look like on ECG?
Rapid, irregular QRS complexes
What can cause early afterdepolarizations?
Hypokalemia
Drugs that prolong QT interval
Are the vast majority of cardiac conditions dominantly or recessively inherited?
Dominantly
When do delayed afterdepolarizations occur?
Phase 4 after repolarization complete but before another action potential would normally occur
What causes delayed afterdepolarizations?
Elevated cytosolic Ca2+ concentrations
What drug toxicity can cause elevated cytosolic Ca2+ concentrations?
Digoxin
What causes the delayed afterdepolarization?
Overload of sarcoplasmic reticulum causes spontaeous Ca2+ release after repolarization
Causes relased Ca2_ to exit cell through 2Na+/2Ca2+ exchanger
Results in net depolarizing current
Bidirectional ventricular tachycardia is a classical feature of what afterdepolarization?
Delayed afterdepolarization
also catecholaminergic polymorphic ventricular tachycardia/CPVT
What causes QT prolongation?
Less repolarising current prolongs APD (action potential duration)
OR
More depolarising current prolongs APD
Polymorphic VT is triggered by what in congenital LQTS?
Adrenergic stimulation
What is the name of the autosomol dominant congenital LQT syndrome that causes isolated LQT?
Romano-Ward syndrome
What is Jervell and Lange-Nielsen-syndrome?
Is it autosomal dominant or recessive?
Long QT syndrome associated with deafness
Autosomal recessive
Are you likely to die from LQT syndrome?
No
What is the treatment for long QT syndrome?
Beta blockers
What should people with LQTS1 avoid?
Strenuous swimming
What should people with LQTS2 avoid?
Exposure to loud noises
How is long QT syndrome diagnosed?
QTc >480ms in repeated 12-lead ECG or LQTS risk score >3
What criteria is used to diagnose LQT syndrome?
Schwartz criteria
What is the criteria for diagnosing congenital short QT syndrome?
QT < 300ms at HR < 80bpm
Congenital short QT syndrome may be associated with what other cardiac condition?
AF
Are you likely to die from congenital short QT syndrome?
Yes
What are some of the problems of implanting a cardioverter defibrillator in children?
Body size
Psychological
Inappropriate discharges due to T wave oversensing
What drugs could you give in congenital short QT syndrome?
Anti-arrhythmic drugs e.g. quinidine, flecainide
What condition causes RBBB in V1-3 and ST elevation?
Brugada syndrome
A patient who has brugada syndrome has an increased risk of developing what arrhythmias?
VT
VF
ECG changes in Brugada syndrome may be intermittent. How can you get a diagnostic ECG in a patient who may have Brugada syndrome?
Provocative testing with flecainide or ajmaline
drugs that block the cardiac Na channel
Why are flecainide and ajmaline used in the provocative testing of a patient with Brugada syndrome?
Block the cardiac sodium channel
What 2 gene mutations are associated with Brugada syndrome?
SCN5A - sodium channel
CACN1Ac - calcium channel
What is the pathophysiology of Brugada syndrome?
Channelopathy
Alters transmembrane ion currents
SCN5A gene codes for cardiac voltage gated Na channel
Defect causes reduced Na current available during phase 0 and early repolarization of cardiac potential
Is Brugada syndrome inheritance mostly dominant or recessive?
Dominant
After provocative drug testing, what would you see on the ECG of a patient with Brugada syndrome?
ST segment elevation with type 1 morphology in 1 or leads (V1 and/or V2)
What can trigger VF in Brugada syndrome?
Rest/sleep
Fever
Excessive alcohol
Large meals
What lifestyle changes should patients with Brugada syndrome make?
Avoid drugs that induce ST segment elevation in right precordial leads
Avoid excessive alcohol + large meals
Prompt treatment of fever
When would you consider implanting an ICD in a patient with Brugada syndrome?
Previous cardiac arrest
Documented sponatenous sustained VT
What drugs would you avoid in Brugada syndrome?
Anti-arrhythmics
Psychotropics
Analgesics
Anaesthetics
What condition is described:
Adrenergic induced bidirectional and polymporphic VT, SVTs?
Catecholaminergic polymorphic ventricular tachycardia
What triggers catecholaminergic polymorphic ventricular tachycardia?
Emotional stress
Physical activity
What can be seen on a patient’s echo and ECG in catecholaminergic polymorphic ventricular tachycardia?
Nothing
Normal
How is catecholaminergic polymorphic ventricular tachycardia investigated?
Autosominal dominant OR recessive (wheyyyyyy u fooled)
What gene mutation is associated with dominant inheritance of catecholaminergic polymorphic ventricular tachycardia (CPVT)?
Ryanodine Receptor mutation (RyR2)
What gene mutatio nis associated with recessive inheritance of catecholaminergic polymorphic ventricular tachycardia?
Cardiac calsequestrin gene (CASQ2)
What lifestyle changes are recommended for patients with CPVT?
Avoidance of competitive sports, strenuous exercise + stressful environments
A patient has recently been diagnosed with CPVT. What drug should they be put on?
Beta blockers
When should you implant an ICD in a patient with CPVT?
If they’ve experienced cardiac arrest, recurrent syncope or polymorphic/bidirectional VT despite optimal therapy
Beta blockers should be considered for genetically positive family members of a person with CPVT despite a negative exercise test. True or false?
True
What should be considered in addition to beta blockers in patients with CPVT that have had recurrent syncope or polymorphic/bidirectional VT whilst on beta blockers (who are not suitable for an ICD)?
Flecainide
What is a last resort therapy for CPVT after drugs and ICD?
left cardiac sympathetic denervation
What is the pathophysiology of Wolff-Parkinson-White syndrome (WPW)?
Presence of accessory pathway between atrium and ventricle
AVRT (atrioventricular re-entrant tachycardia) is the most common arrhythmia in what condition?
WPW
Squatting babies are associated with what cardiac condition?
Tetralogy of fallot
What are the 4 components of tetralogy of fallot?
Pulmonary stenosis
Ventricular septal defect
Right Ventricular hypertrophy
Overriding aorta
A mutation in what genes causes hypertrophic cardiomyopathy?
Sarcomeric genes (MYH7/MYBPC3)
How does hypertrophic cardiomyopathy tend to present? (4)
Sudden death
Heart failure
End stage heart failure
AF
What is the treatment for hypertrophic cardiomyopathy?
ICD if severe (prior cardiac arrest/sustained VT) or lotsa risk factors (FHx in first degree relative, LV wall thickness >30mm, recent unexplained syncope)
What can you use if you are unsure of a patient’s risk of sudden cardiac death?
HOCM: SCD risk calculator
What lifestyle modifications should people with HOCM make?
Avoid competitive sports
What genes cause dilated cardiomyopathy?
Sacromere + desmosomal genes
laminA/C and desmin if conduction disease
Dystrophin if X-linked
Who tends to get dilated cardiomyopathy, males or females?
Males
not common in childhood
Describe the pathogenesis of arrhythmogenic right ventricular cardiomyopathy
Fibro-fatty replacement of cardiomyocytes
What autosomal dominnat mutations cause arrythmogenic right ventricular cardiomyopathy?
Desmosomal proteins
What autosomal recessive mutations cause arrhythmogenic right ventricular cardiomyopathy?
Nondesmosomal genes
What arrhythmia can be caused by arrhythmogenic right ventricular cardiomyopathy?
VT
What lifestyle modifications are recommended in arrhythmogenic right ventricular cardiomyopathy?
Avoidance of competitive sports
What is the first line therapy in arrhythmogenic right ventricular cardiomyopathy?
Beta blockers
What drug can be used in patients intolerant of beta blockers in arrhythmogenic right ventricular cardiomyopathy?
Amiodarone
Is ICD used in arrhythmogenic right ventricular cardiomyopathy?
YEah
In patients with history of aborted SCD and haemodynamiclaly poorly tolerated VT
What is the only known treatment for VF?
Defibrillation
What does defibrillation do?
Delivers high energy to all cardiac myocytes causing them to fully depolarise (resets them)
Lamin A/C mutations are associated with which inherited condition?
Dilated cardiomyopathy
First degree heart block
SVT and VA
What is the treatment for all patients with DCm and confirmed disease-causing LMNA mutation and risk factors?
ICD