[SCD-FORUM] 143E Seven year old boy with suspected LQT3 Dr. Costa

[SCD Symposium]

Dear collegues:
A seven year old boy was admitted 1 week ago because he had two episodes of syncope at rest, last one with seizures; after a neurological evaluation, a ECG was performed , and showed a QTc prolongation of 520 msec, with late onset peacked/biphasic T wave pattern, resembling a LQT3. His father and two sisters, of 12 and 14 years old, has long QT >500 msec. All three are still asymptomatic and had a non characteristic pattern of LQT3. His mother, who has a normal ECG, has a history of two brothers with sudden death at ages 25 and 28. Since the first day of hospitalization no ventricular arrhythmia was seen.
Treatment with a beta blocker at regular dosage had no effect on QTc duration. Three days ago we began with the administration of oral flecainide, and the QTc measure is now 540 msec. I would like to ask the experts if this patient is at high risk of sudden death, and if it is true, do you suggest an ICD implantantion?
Thank you in advance
Dr. Gustavo Costa, MD 
Laboratorio de Electrofisiología Hospital Nacional Alejandro Posadas
Buenos Aires - Argentina