Cardiac Causes of Sudden Death Flashcards
What are the three broad categories of inherited cardiac conditions that may present as sudden death?
inherited arrhythmia syndromes
Inherited cardiomyopathy
Inherited multisystem diseases with CVS involvement (e.g. myotonic dystrophy)
what would ECG screening pick up?
channelopathies
cardiomyopathies
triggered activity of the heart may be caused by…
after deoplarizations
a longer QT interval means a higher chance of what?
an extra depolarization
Syndrome caused by a sodium channel problem?
Brugada syndrome
which syndrome is very rare and people with it usually die in childhood?
Short QT syndrome
how many people are carriers of a long QT gene?
1/2000
what can trigger polymorphic VT (Torsades de pointes)?
adrenergic stimulation
what is the risk of sudden death in untreated long QT syndrome?
under 1%
What proportion of people with long QT experience syncope episodes annually?
5%
how high would your risk of cardiac arrest from long QT syndrome need to be to have an ICD?
4%
what scoring system is used to calculate likelihood of LQTS?
schwartz score
what are some lifestyle changes to make to prevent an event with LQTS?
avoid OT prolonging drugs
correct electrolyte abnormalities (e.g. when have diarrhoea, vomiting etc.)
avoid genotype specific features- some triggered by strenuous exercise/ loud noises e.g. alarm clocks)
What does Brugada syndrome put you at risk of?
polymorphic VT or VF
what does Brugada syndrome look like on ECG?
ST elevation in RBBB in V1,2,3
females are 8X more likely to get brugada syndrome TRUE/ FALSE
FALSE
males
what triggers Brugada syndrome?
rest/sleeping
fever
ST elevating drugs
excessive alcohol/food
what is the incidence of arrhythmia from Brugada syndrome in asymptomatic people?
1%
what is the incidence of arrhythmia from Brugada syndrome in patients with syncope?
3.2%
what is the incidence of arrhythmia from Brugada syndrome in patients who have previously had a cardiac arrest?
13.5%
If a person with Briugada syndrome is coming down with a fever, what must they do?
take early paracetamol!!!
What is CPVT?
chatecholaminergic polymorphic ventricular tachycardia
describe the pathophysiology of CPVT
abnormal response to adrenaline.
problem in RYANODINE receptor
what arrhythmia can be a result of CPVT?
Bi-directional VT
Name two treatments for CPVT?
beta blockers- v tight control
ICD
what is the most common arrhythmia with Wolff-Parkinson White Syndrome?
Atrioventricular re-entrant tachycardia
how does WPW show on ECG?
short PR interval and delta wave
where is the mutation in 60% of hypertrophic cardiomopathies?
sarcomere genes
what is a big trigger of sudden death from hypertrophic cardiomyopathy?
strenuous exercise
dilated cardiomyopathies are more common in males, true/false?
TRUE
which cardiomyopathy involves fibro-fatty replacement of cardio myocytes?
arrhythmogenic right ventricular cardiomyopathy
what mode of inheritance is ARVC usually?
autosomal dominant
