arrhythmias Flashcards

1
Q

channelopathies

A

problem in conducting system usually due to protein problems that predispose to rhythm problems

arrhythmogenesis is related to ion current imbalance + development of early + late depolarisations

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2
Q

examples of channelopathies

A

congenital long QT syndrome
brugada syndrome
catecholaminergic polymorphic ventricular tachycardia (CPVT)
short QT syndrome
progressive familial conduction disease
familial AF
familial WPW

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3
Q

cardiomyopathies

A

arrythmogenesis is related to scar/electrical barrier formations + subsequent re-entry

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4
Q

examples of cardiomyopathies

A

hypertrophic cardiomyopathy
arrhymogenic right ventricular cardiomyopathy (ARVC)
dilated cardiomyopathy

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5
Q

what causes early after depolarisations (EADs)?

A

increase in frequency of abortive action potetials before normal repolarisation is completed

can result in Torsades de Pointes

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6
Q

when do early after depolarisations occur?

A

occur with abnormal depolarisations during phase 2 or phase 3

  • phase 2 may be interrupted due to augmented opening of Ca channels
  • phase 3 interruptions are due to opening of sodium channels
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7
Q

what can early after depolarisations (EADs) be potentiated by?

A

hypokalamia
drugs that prolong QT interval - amiodarone, sotalol, haloperidol, erythromycin, citalopram

(EADs can result in Torsades de Pointes)

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8
Q

what are delayed after depolarisations caused by?

A

elevated cystolic calcium concentrations
- classically seen with digoxin toxicity

overload of sarcoplasmic reticulum may cause spontaneous Ca2+ release after repolarising
- causing the released Ca2+ to exit the cell through 3Na+/Ca2+ exchanger -> nedepolarising current

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9
Q

classic feature of delayed after depolarisations

A

bidirectional ventricular tachycardia
- also seen in catecholaminergic polymorphic ventricular tachycardia (CPVT)

(CPVT - cardiomyocytes are hypersensitive to adrenaline + other caracholamines)

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10
Q

when do delayed after depolarisations occur?

A

begin during phase 4 after repolarisation but before another action potenitial would normally occur via normal conduction systems of heart

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11
Q

biggest complication of congenital long QT syndrome

A

risk of polymorphic VT (torsades de pointes) triggered by adernergic stimulation

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12
Q

risk of sudden cardiac death in long QT syndrome

A

0.33-0.9%

risk of this happening is related to
- legth of prologations - longer= higher risk
- pre-adolescent males
- adult female
- prior syncope
- response to beta blockers - if respond well risk low

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13
Q

long QT subtypes

A

13 subtypes
- different types appear differently on ECG, different penetrance

  • autosomal dominant - isolated LQT or extra cardiac features
  • autosomal recessive - assoc with deafness
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14
Q

long QT syndrome diagnosis

A

QT >=480ms in repeated ECGs

LQTS risk score >3

-> confirmed pathogenic LQTS mutations irrespective of QT duration

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15
Q

management of long QT syndrome

A

long acting beta blocker (NOT SOTALOL)
lifestyle
- avoid QT prolonging drugs
- correct electrolyte abnormalities
- avoidance of genotype specific triggers

LQTS1 - strenous swimming
LQTS2 - exposure to loud noises

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16
Q

torsades de pointes

A

polymorphic ventricular tachycardia
occurs in patients with prolonged QT interval

will either terminate spontaneously + revert back to sinus rhythm or progress in to ventricular tachycardia which can lead to cardiac arrest

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17
Q

prolonged QT interval

A

ECG finding of prolonged repolarisation of the muscle cells in the heart after contractions

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18
Q

depolarisations

A

electrical process that leads to heart contractions

19
Q

repolarisation

A

period of recovery before myocytes are ready to depolarise again

20
Q

physiology of torsades de pointes

A

waiting a longer time for repolarisation can result in random spontaneous depolarisation in some areas of myocytes
- these abormal spontaneous depolarisations prior to repolarisation are known as “after depolarisations”

these spread throught the ventricle, leading to ventricular contraction prior to proper repolarisation occuring
- when this occurs + the ventricle continue to stimulate recurrent contractions without normal repolarisation = Torsades de pointes

21
Q

causes of prolonged QT

A

long QT syndrome (inherited)
medications
electrollyte disturbances

22
Q

what medications can cause prolonged QT syndrome

A

antipsychotics
citalopram
flecanide
sotalol
amiodarone
macrolide antibiotics

23
Q

what electrolyte disturbances can cause long QT syndrome

A

hypOkalaemia
hypomagnesaemia
hypocalcaemia

24
Q

management of torsades de pointes

A

acute
- correct cause - electrolyte/medication
- magnesium infusion - even if normal serum magnesium
- defibrillation if VT occurs

long term mx of prolonged QT
- avoids QT long meds
- beta blocker - NOT sotalol
- pacemaker or implantable defibrillator

25
congenital short QT syndrome
very rare caused by mutation in cardiac K+ channels may be assoc with AF may present in young kids - most dont survive very malignant
26
brugada syndrome
inherited channelopathy risk of polymorphic VT, VF AF commoon 12 assoc genes autosomal dominant adults, 8x more common in males also if previous cardiac arrest
27
ECG changes seen in brugada syndrome
ST elevation + RBBB in V1-V3 ECG finfing may be intermittent + change over time changes may only be seen with provocative testing -> flecanide or ajmaline - drugs that block the cardiac sodium channel
28
VF triggers in brugada syndrome
usually rest or sleep fever excessive alcohol, large meals genotype + fam history of sudde cardiac death DOES NOT influence prognosis
29
management of brugada syndrome
lifestyle - avoid drugs that induce ST elevation in right leads -- anti-arrhythmis drugs -- psychotropics -- analgesics -- anaesthetics - avoid excessive alcohol + large meals - prompt treatment of any fever with paracetamol ICD implantation if - survived aborted cardiac arrect - documented spontaneous sustained VT - considered is spontaneous type I ECG pattern + history of syncope
30
catecholaminergic polymorphic ventricular tachycardia (CPVT)
adrenergic induced bidirectional + polymorphic VT, SVTs triggered by emotional stress of physical physical activity 1 in 1000 at rest, normal ECG + ECHO autosomal dominant + recessive
31
CPVT management
lifestyle - avoid competitive sports, strenous exercise, stressful environments beta blockers for all - consider for genetically positive family members even after negative exercise test ICD implantation +/- flecainide - for patients who experient recurrent syncope, cardiac arrest, polymorphic/bidirectional VT despite optimal therapy
32
Wolff-Parkinson White syndrome
accessory pathway connecting atria to ventricles (bundle of kent) - usually only one pathway -> SA node small risk of sudden cardiac death (SCD)
33
management of Wolff-parkinson white
radiofrequency ablation of accessory pathway
34
hypertrophic cardiomyopathy
common mutation in sarcomere gene that codes for development of cardiac muscle
35
causes of hypertrophic cardiomyopathy
40-60% sarcomeric protein gene mutation Anderson-Fabry mitochondrial diseases neuromuscular disease amyloidosis new-born of diabetic mother drug induced - tacrolimu, hydroxycholoquine, teroids
36
hypertrophic cardiomyopathy presentation
varies massively sudden death heart failure end stage heart failure atrial fibrillation syncope following exercise jerky pulse, double apex beat ejection systolic murmur
37
prevention of sudden cardiac death in hypertrophic cardiomyopathy
avoid competitive sports ICD implantation in patient who have survived cardiac arrest due to VT or VF evaluate 5yr risk every 1-2yrs
38
arrhythmogenic right ventricular cardiomyopathy (ARVC)
occurs when fibro-fatty replacement of cardiomyocytes - fatty deposits in RV wall - current travels around the fat causing reentry VT LV involvement in >50%
39
inheritance of arrhythmogenic right ventricular cardiomyopathy (ARVC)
autosomal dominant mutations in genes from desmosomal proteins autosomal recessive mutation in nondesmosomal genes around 50% of patients have a mutation of one of the several genes which encode components of desmosome (variable expression)
40
management of ARVC
1st - beta blockers(sotalol) - titrated to max tolerated dose - amiodarone if not tolerated ICD implantation catheter ablation avoid competitive sports ICD depends on risk of SCD - fam history of premature SCD - severity of RV + LV function - frequent non-sustained VT - ECG = QRS widening - male - age of presentation
41
investigations of ARVC
ECG - abnormalities in V1-3, typically T wave inversion - epsilon wave is found in about 50% - best described as a terminal notch in the QRS complex ECHO - often subtle in early stages but may show an enlarged, hypokinetic right ventricle with a thin free wall MRI useful to show fibrofatty tissue
42
drugs to avoid in hypertrophic cardiomyopathy
nitrates ACEi inotropes
43
features of dilated cardiomyopathy
classic findings of heart failure systolic murmur - stretching of valves may cause mitral + tricuspid regurgitation S3 "balloon" appearance on CXR