[SCD-FORUM] 17S RE: A children with LQTS. Dr. Arabia

[SCD Symposium]

Dear Dr. Haghjoo,
We agree with you in that your patient is in increased risk of sudden  
death by the factors you point out.
The fact that she repeated a syncope event in spite of 30 mg of  
propranolol in a 5-year-old child (although we ignore her weight),  
with a heart rate of 55 with clear signs of beta block, suggests at  
least unsatisfactory response to such medication.
In such a case, we would rather implant an ICD, knowing as a  
pediatric electrophysiologist, what this decision implies. We would  
attempt the implantation using an endocardial electrode/coil, but  
implanted epicardially, surrounding the LV epicardium and the  
inferior side of the RV. The ICD may even be a dual-chamber one, and  
an atrial epicardial electrode may be implanted in the RA, attempting  
to shorten the QT with increase of HR, but allowing intrinsic  
conduction.
The aggressiveness in the management is due to the fact that the next  
event may be the last in this disease.
Congratulations to the organizers of this symposium for their  
excellent work.

Sincerely,

Luis Arabia MD.
Córdoba
Argentina

--
Dr. Sergio Dubner
President of Scientific Committee

Dr. Edgardo Schapachnik
President of Steering Committee

>
> Dear Dr. Dubner,
>
> Thank you and your colleagues for this nice online symposium.
> Recently, a
> 5-year-old children with no evidence of structural heart disease
> referred to
> our center for evaluation of syncope. He also had history of  
> congenital
> deafness. One of the episodes of syncope occurred 48-hrs after
> betablocker
> withdrawal and second episodes on 10 mg propranolol tid. Surface ECG
> revealed a QTc=600 ms and heart rate of 55 bpm. We think that onset in
> childhood, congenital deafness and QTc=600 would place this patient at
> higher risk for cardiac events and therefore a candidate for ICD
> implantation. However, small body size and future risk of  
> inappropriate
> therapy with its devastating consequences are unfavorable aspects  
> of ICD
> implantation. Our limitimg factors for increase in dose of
> betablocker is
> low baseline hear rate. What is recommendation of scientific
> committee of
> SCD symposium regarding our patient?
>
> Thank you in advance for your recommendation.
>
> Regards,
>
> Majid Haghjoo,MD
> Department of Pacemaker and Electrophysiology
> Rajaie Cardiovascular Medical and Research Center
> Tel: +98 21 2392 2931
> Fax:+98 21 2204 8174
> Tehran, Iran
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