[SCD-FORUM] 16E RE: A children with LQTS. Dr. Towbin

[SCD Symposium]

We would place an epicardial lead with a single chambered ICD in this  
child with probable Jervell-Lange-Nielsen syndrome.We would not be  
deterred by the age or size of the child.

Jeffrey Towbin, MD
Professor and Chief
Pediatric Cardiology
Texas Children's Hospitasl
Baylor College of Medicine
Houston, Texas USA

--
Dr. Sergio Dubner
President of Scientific Committee

Dr. Edgardo Schapachnik
President of Steering Committee


>
> By our experience of the managment and follow up of children with  
> congenital
> LQTS, beta blockers (Propranolol, Atenolol, Nadolol) therapy in  
> daily dose
> 1-2 mg/kg not significantly increase of bradycardia in this pts.  
> Sometimes
> combination of the regular doses of betablockers (1 mg/kg) and Na  
> blockers
> (we use carbamazepin 10 mg/kg/daily) may be useful. But in this  
> case patient
> had syncope, very long QTc (600 ms) and heart rate 55 bpm for 5  
> years old
> children on rest ECG  is very low.  By my opinion children need in
> antyarhythmic device in combination with drug therapy.
> Dr. Leonid Makarov
> Moscow Institute pediatry and children surgery
> Moscow, Russia. Taldomskaya str. 2
> leo at oss.ru
>
> --
> 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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