congenital long QT syndrome

Question 1

csued by

Answer 1

drugs or conditions that prolong ventricular repolarisation

Question 2

autosomal dominant forms

Answer 2

isolated LGT - romano ward syndrome

extra cardiac feature - Anderson Tawil and timothy

Question 3

autosomal recessive forms

Answer 3

Jervell and Lange Neilsen

Question 4

autosomal recessive forms associated with

Answer 4

deafness

Question 5

how is QT segment prolonged

Answer 5

inc in depolarising current (Na and Ca) and dec in repolarising current (K)

Question 6

caused by mutations in which genes

Answer 6

KvLGT1 or HERG (K) channels

SNC5A (Na channels)

Question 7

ECG causes

Answer 7

polymorphic ventricular tachycardia

Question 8

can cause what type of polymorphic ventricular tachycardia

Answer 8

torsades de pointes

Question 9

torsades de pointes can lead to

Answer 9

AF and sudden death

Question 10

diagnosis

Answer 10

12 lead ECG or LQTS risk score > 3

or presence of confirmed pathogenic LQTS mutation