Long QT syndrome

Question 1

What is long QT syndrome?

Answer 1

Describes an ECG where the ventricular repolarisation (QT interval) is greatly prolonged.

Question 2

Congenital aetiology of long QT syndrome?

Answer 2

Jervell-Lange-Nielson syndrome (autosomal recessive) - associated with deafness

Romano-ward syndrome (autosomal dominant)

Question 3

Acquired aetiology of long QT syndrome?

Answer 3

Electrolyte abnormalities, drugs, diabetes and acute MI.

Question 4

What are drug trigger examples of acquired long QT syndrome?

Answer 4

Amiodarone

Tricyclic antidepressants

Antibiotics

Fluconazole

Erythromycin

Haloperidol

SSRI’s (selective serotonin re-uptake inhibitors)

Question 5

Clinical presentation for long QT syndrome?

Answer 5

Patients with a long QT develop syncope and palpitations due to polymorphic ventricular tachycardia (torsades de pointes).

Usually terminates spontaneoiusely however can degenerate to ventricular fibrillation, resulting in sudden death.

Question 6

Long term management for congenital QT?

Answer 6

Beta blockers - very effective at reducing sudden cardiac death.

Avoid QT prolonging drugs and other triggers

ICD