[SCD-FORUM] 22E RE: A children with LQTS. Dr. Dubner

[SCD Symposium]

Dear Dr. Majid Haghoo
You describe a difficult situation and I am not a pediatric specialist.
Certainly, with a QTc larger than 500 msec and congenital deafness  
your patient is at high risk. If the child was older, an ICD should  
be implanted. At 5 years of age, the ICD could be more of a problem  
then a benefit.
There are many problems with ICD implantation and maintenance in this  
group of patients. Under the special circumstances it is warranted,  
and possibly using a few different implant techniques. So certainly,  
recurrence of LQT symptoms on meds in this child would be an  
indication. If necessary and according with world experts, I'd  
probably try a subcutaneous coil in the back with an abdominal can.
On the other hand, it seems to me that a genetic test should be  
important in this young patient. Identifying the genetic mutation  
could guide you with the pharmacological treatment.
You should contact Arthur Moss, MD at Rochester University  
(Rochester, NY) to include your patient in the LQTS registry and make  
the test and Philip Saul, MD at Medical University of South Carolina  
(Charleston, SC) for surgical techniques.
Regards

Dr. Sergio Dubner

--
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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