Cardiology 1 Flashcards
(110 cards)
What is a channelopathy?
A genetic disorder in which the electrical activity of the heart is abnormal, but the heart is structurally normal.
When do symptoms first appear in channelopathies?
30-40 years
What is the cause of Brugada syndrome, a type of channelopathy?
Cardiac sodium channelopathy (SCN5A gene)
Autosomal dominant inheritance but can be a new mutation.
How does Brugada syndrome cause arrhythmias?
Abnormally slow conduction in the heart - decreased flow of Na+ ions changes the AP, slowing conduction.
“Short circuits” form, causing wavebreak and causing re-entry (U-turns).
What are the symptoms of channelopathies?
May be asymptomatic Syncope Sudden cardiac death Palpitations Chest pain Breathlessness or dizziness
How is syncope caused by a channelopathy?
Brief arrhythmias revert to a normal rhythm spontaneously OR vasovagal syncope.
In Brugada syndrome, when do arrhythmias occur?
At rest
Following a heavy meal
Fever/alcohol
When vagus nerve is activated
How is Brugada syndrome diagnosed?
Genetic testing
ECG with or without flecainide (provocation test):
Brugada sign: coved ST segment elevation >2mm in >1 of V1-V3 followed by a negative T wave
What must Brugada sign be associated with to diagnose channelopathies?
One of:
- Documented VF or polymorphic VT
- FH of SCD<45 years
- FH of similar ECG
- Syncope
Which arrhythmias may be caused by a Brugada syndrome?
Ventricular fibrillation
Polymorphic ventricular tachycardia
AV nodal re-entrant tachycardia
Sinus node dysfunction
As part of the treatment of Brugada syndrome, what triggers must be avoided?
Fever Alcohol and drugs Dehydration Tricyclics, anaesthetics, sodium channel blockers (flecainide) CCBs
What is the main treatment of Brugada Syndrome?
Implantable cardiac defibrillator
If not appropriate - quinidine
What is a normal QT interval?
<440ms (two large squares)
What are the risks of prolonged QT syndrome?
Delayed repolarization of the heart following a heart beat increases the risk of toursades des pointes episodes
What are the congenital causes of LQTS?
Cardiac ion channel mutation:
Jervell and Lange Nielson syndrome
Romano-Ward syndrome
What are the acquired causes of LQTS?
Low Ca/K/Mg Drugs: amiodarone, TCAs, SSRIs MI 3rd degree heart block Fasting and bradycardia
What are the subtypes of LQTS?
Potassium channel: risk of SCD when patient is startled or awoken suddenly
Sodium channel: risk of SCD when patient is sleeping
What are the symptoms of LQTS?
Syncope and palpitations as a result of polymorphic VT
Nausea, pallor, dyspnoea, chest pain
What is seen on the ECG in LQTS?
QTc>440m/s
Toursades des points
Degeneration into sustained VT or VF
What is the treatment of congenital LQTS?
Propanolol (unless bradycardic)
Pacing
ICD
Why is propranolol contra-indicated in acquired LQTS?
Bradycardia can precipitate toursades.
What is the treatment of toursades des pointes?
IV magnesium sulphate
Temporary atrial pacing to increase HR and reduce QT
Correct predisposing factor e.g. QT prolonging drug
IV isoprenaline
Define cardiomyopathy.
Myocardial disorder in which the heart muscle in structurally and functionally abnormal without coronary artery disease, hypertension, valvular, or congenital heart diseases.
How can cardiomyopathies present?
Heart failure
Arrhythmias
SCD
Thromboembolism