A 16 year old girl in cardiology clinic suddenly collapsed after listening fire alarm in hospital

A 16 year old girl accompanying his father in cardiology clinic suddenly collapsed after listening fire alarm in hospital. Senior cardiology fellow successfully resuscitated her. On recovery her physical examination was normal. On inquiry his father informed that she fainted twice at home after doorbell with spontaneous recovery.

a) What do you think happened to this young girl?

b) Describe two characteristic ECG findings

c) How will you manage her?

d) Which genetic mutation cause this disease?

e) Is flecainide indicated?

Answers:

a.      Polymorphic VT (Underlying long QT type II)

b.      QTc > 480 msec, Low amplitude notched T wave

c.       Beta-blockers (Propanolol, Nidolol) and ICD if recurrent syncope despite on beta blockers or if contraindication to beta blocker and in survivors of cardiac arrest

d.      KCNH2

e.      No

Long QT types

References:

1. 2015 ESC Guidelines for management of patients with ventricular arrhythmia and prevention of SCD

2. 2017 ACC/AHA/HRS guidelines for evaluation of patient with syncope

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