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Cardiovascular > Inherited Cardiac Conditions > Flashcards

Inherited Cardiac Conditions Flashcards

1
Q

what can an arrhythmia be?

A

a common manifestation of many genetic conditions
inherited arrhythmia syndromes
inherited cardiomyopathies
inherited multi-system diseases with CVS involvement
myotonic dystrophy

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2
Q

2 types of arrhythmogenic inherited cardiac conditions

A

channelopathies

cardiomyopathies

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3
Q

channelopathies examples

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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4
Q

cardiomyopathies examples

A

hypertrophic cardiomyopathy
arrhythmogenic right ventricular cardiomyopathy
dilated cardiomyopathy

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5
Q

channelopathies

A

arrhythmogenesis is related to ion current imbalance and development of early and late depolarisations
transmural ADP dispersion

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6
Q

cardiomyopathies

A

arrhymogenesis related to scar/electrical barrier formation and subsequent re-entry

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7
Q

summation of all ion currents across the cell membrane

A

the surface ECG

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8
Q

after depolarisations

A

abnormal depolarisations of cardiac myocytes that interrupt phase 2,3 or 4 of the cardiac AP
can lead to triggered activity seen as sustained cardiac arrhythmia

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9
Q

when are early afterdepolarisations?

A

during phase 2 or 3

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10
Q

what causes early afterdepolarisations

A

an increase in frequency of abortive APs before normal repolarisation is completed

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11
Q

what can EADs lead to?

A

torsades de pointes

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12
Q

why would phase 2 be interrupted by an EAD?

A

augmented opening of calcium channels

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13
Q

why would phase 3 be interrupted by an EAD?

A

opening of sodium channels

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14
Q

when do delayed afterdepolarisations occur?

A

during phase 4, after repolarisation is completed but before another action potential would normally occur via the normal conduction system of the heart

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15
Q

why do DADs occur?

A

due to elevated cystolic calcium concentrations
the overload of the SR may cause spontaneous Ca2+ release after repolarisation, causing the released Ca2+ to exit the cell through the 3Na+/Ca2+ exchanger
this results in a net depolarising current

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16
Q

when are DADs seen?

A

digoxin toxicity
bidirectional ventricular tachycardia
catecholaminergic polymorphic ventricular tachycardia (CPVT)

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17
Q

what is the mechanism of QT prolongation?

A

less repolarising current prolongs ADP
-or-
more depolarising current prolongs ADP

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18
Q

congenital LQTS

A 
polymorphic VT (torasades de pointes) triggered by adrenergic stimulation
syncope
risk associated with severity of QT prolongation
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19
Q

autosomal dominant LQTS

A

isolated LQT- romano-ward syndrome

extra cardiac features- anderson-tawil syndrome, timothy syndrome

20
Q

autosomal recessive LQTS

A

associated with deafness; jervell and lange-neilsen syndrome

21
Q

LQTS diagnosis

A

QTc >480 ms in repeated 12-lead ECGs or LQTS risk score >3

confirmed pathogenic LQTS mutation irrespective of the duration

QTc >460 ms in repeated 12-lead ECGs in patients with an unexplained syncopal episode in the absence of secondary causes for QT prolongation

22
Q

risk of sudden cardiac death in LQTS

A 
age dependant 
gender 
- pre-adolescent males
- adult females
increasing QT duration 
prior syncope 
response to beta blockers
23
Q

risk management in LQTS

A

avoidance of QT prolonging drugs
correction of electrolyte abnormalities
avoidance of genotype-specific triggers eg strenuous swimming and exposure to loud nosies

24
Q

brugada syndrome

A

risk of polymorphic VT, VF
atrial fibrillation common
ST elevation and RBBB in V1-V3
ECG findings may be intermittent and change over time
diagnostic ECG changes may seen only with provocative testing with flecainide or ajmaline
autosomal dominant
usually adult males

25
Q

VF triggers in brugada syndrome

A

rest or sleep
fever
excessive alcohol, large meals

26
Q

risk management in brugada syndrome

A

avoidance of drugs that may induce ST segement elevation in right precordial leads
avoidance of excessive alcohol intake and large meals
prompt treatment of any fever with antipyretic drugs

27
Q

ICD implantation in brugada syndrome

A

survivors of cardiac arrest or have documented sustained VT

spontaneous diagnostic type I ECG pattern and history of syncope

28
Q

drugs to avoid in brugada syndrome

A

anti-arrhythmic drugs
psychotropics
analgesics
anaesthetics

29
Q

catecholaminergic polymorphic ventricular tachycardia

A

adrenergic induced bidirectional and polymorphic VT, SVTs, triggered by emotional stress, physical activity
normal ECG and ECHO

30
Q

autosomal dominant catecholaminergic polymorphic ventricular tachycardia

A

ryanodine receptor mutation

31
Q

recessive catecholaminergic polymorphic ventricular tachycardia

A

cardiac calsequestrin gene

32
Q

CPVT risk management

A

avoidance of competitive sports, strenuous exercise and stressful environments
B blockers

33
Q

ICD implantation in CPVT

A

cardiac arrest
recurrent syncope
polymorphic/ bidirectional VT despite optimal therapy

34
Q

flecainide

A

patients with CPVT where there are risks/contraindications for an ICD

35
Q

complications associated with transvenous leads

A 
endocarditis 
perforation
haemothorax
pnemothorax
thromboembolic events
vascular complications 
lead fractures
lead extraction complications 
lead dislodgement
36
Q

S-ICD

A

an alternative to transvenous defibs in patients with an indication for an ICD when pacing therapy for bradycardia, cardiac resynch or antitachycardia pacing is not needed

37
Q

hypertrophic cardiomyopathy

A

mutation in sarcomeric genes

38
Q

HOCM clinical presentations

A

sudden death
heart failure end stage HF
atrial fibrillation

39
Q

dilated cardiomyopathy

A

more males than females

sarcomere and desosomal genes/ laminA/C and desmin if there is conduction disease, dystophin if X linked

40
Q

lamin A/C

A 
progressive dilated cardiomyopathy
atrioventricular block (first degree heart block) 
SVT and VA 
high risk of sudden death
sometimes neuromuscular symptoms
41
Q

arrhythmogenic right ventricular cardiomyopathy

A

fibro-fatty replacement of cardiomyocytes

LV involvement

42
Q

mutations in arrhythmogenic right ventricular cardiomyopathy

A

autosomal dominant in the genes for desmosomal proteins

autosomal recessive mutations in nondesmosomal genes

43
Q

risk of sudden death in ARVC

A 
family history 
severity of RV and LV function 
frequent non-sustained VT 
QRS prolongation 
VT induction on EPS 
male 
age
44
Q

risk management in ARVC

A 
avoidance of competitive sports 
B blcokers 
ICD implantation 
amiodarone 
catheter ablation
45
Q

cascade screening

A

Produces a greater rate of case identification than general population screening.
Once a diagnosis is confirmed in an individual, testing is extended to first degree and second degree relatives.
If relatives test positive,
their first and second degree
relatives are approached and
offered testing, and so on

Cardiovascular (31 decks)

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