Inherited Cardiac Conditions

Question 1

What is the management of long QT syndrome?

Answer 1

avoid QT prolonging drugs, correction of electrolyte imbalance, avoidance of triggers

Question 2

What triggers polymorphic VT/Torsades de Pointes in those with Brugada syndrome?

Answer 2

sleep, fever, excessive alcohol, large meals

Question 3

Is Brugada syndrome more common in males or females?

Answer 3

males

Question 4

What is long QT syndrome?

Answer 4

QT interval >440ms in males and >450ms in females

Question 5

How is Brugada inherited?

Answer 5

autosomal dominant

Question 6

What are the clinical features of Brugada syndrome?

Answer 6

polymorphic VT/Torsades de Pointes, VT, AF

Question 7

What triggers Torsades de Pointes/polymorphic VT in those with long QT syndrome?

Answer 7

adrenergic stimulation - exercise, sudden auditory stimuli, breath holding, sleep, hypokalaemia

Question 8

What drugs should be avoided in Brugada syndrome?

Answer 8

anti-arrhythmic, psychotropics, analgesics, anaesthetics

Question 9

What are the risks of long QT syndrome?

Answer 9

sudden cardiac death, Torsades de Pointes/polymorphic VT, syncope

Question 10

What is seen in an ECG of someone with Brugada syndrome?

Answer 10

ST elevation, right bundle branch block in V1-V3

Question 11

What causes Wolff-Parkinson White syndrome?

Answer 11

congenital accessory conduction pathway between the atria and ventricles

Question 12

What are the possible ECG features of Wolff-Parkinson White syndrome?

Answer 12

short PR interval, wide QRS complex with a slurred upstroke - delta wave, left axis deviation if right-sided accessory pathway, right axis deviation of left-sided accessory pathway

Question 13

What are the complications of Wolff-Parkinson White syndrome?

Answer 13

atrioventricular re-entry tachycardia, accessory pathway doesn’t slow conduction - AF can degenerate rapidly to VF