[SCD-FORUM] 27E RE: A children with LQTS. Dr. Furlani

[SCD Symposium]

Dear Dr Haghjoo:
This a difficult case, which sounds like Jervell and Lange-Nielsen  
Syndrome. The patient is a 5 years old kid what makes this case even  
more difficult. No perfect options/treatment strategies are available  
in dealing with patient yet.
Dr Peter Schwartz and the international registry for LQTS have helped  
us to start to understand this life threatening disease and learn how  
to manage this patients, but there is still a long way to go.
In this case, the early onset of the symptoms, presence of congenital  
deafness, severity of QT prolongation (over 500 msecs), and lack of  
response (or parcial response) to beta-blocker therapy, are all  
markers of high risk for SCD. On the other hand, as you pointed out  
in your e-mail, ICD implantation in children is associated with  
important colateral problems, which should be kept in mind in  
considering this therapeutic option.
Different options come to my mind in dealing with this patient:
1) Increase the dosage of propranolol (or change it for 1 mg/Kg of  
Nadolol Die) even in the presence of mild bradycardia. In this  
patients we can tolerate until 40 bpm while they are asymptomatic or  
mildly symptomatic.
2) Left cardiac sympathetic denervation plus Nadolol 1 mg/Kg Die.
3) ICD implantation if the patient has another episode of syncope  
after option 2.
4) Of course, all viable options have to include limitation of  
physical activity since this patients usually die while exercising  
and swimming is particularly hazardous.
Genotyping the patient is not so useful in this case since most of  
the JLN patients have KvLQT1 (LQT1)  and KCNE1 (LQT5) abnormalities.
Hopefully, in the future gene therapy will be the first therapeutic  
option to actually cure these patients.
Thank you.
My best regards for everybody,

Aldo Alberto Furlani MD
Cardiac electrophysiologist
Consultant Cardiologist
Heart Institute of the Caribbean
Mandeville
Jamaica
afurlani at caribbeanheart.com
www.caribbeanheart.com

--
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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> Scd-forum at scd-symposium.org
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