[SCD-FORUM] 45C RE：关于5岁LQTS患者的答复Cuilan Li

[SCD Symposium]

亲爱的Haghjoo医生：你好！
治疗这样年幼的LQTS患者对医生来讲确实是一个挑 
战，尤其在患者心律较慢，倍他阻滞剂用量加不上去 
的时候。基于我们研究组的经验，我的建议是：
1． 将倍他阻滞剂普萘洛尔换成长效美托洛尔或纳多 
洛尔（nadolol），取决于在你的国家你能够得到那种 
药物。

2． 如果患者在服药期间还有晕厥发作，建议她进行 
左心交感神经切除手术。我们在5年前曾为一个6岁男 
孩进行手术，去年又为一个7岁女孩进行过手术。与 
给成年人手术相比手术有一定难度，但不是不可以克 
服。Schwartz教授的研究组在这方面有更多的经验。

3． 当患者的年龄和身体体积足够大时，视症状情况 
可以再考虑ICD。


在这样的会议上和来自世界各地的专家交流观点是一 
种全新美好的体验，感谢会议组织者为我们提供这样 
的机会，也感谢来自世界各地众多的参与者！

李翠兰
北京大学人民医院心内科
北京 100044, 中国
Tel: 86-10-68314422 Ext. 5940
licuilan在gmail.com


Dear Dr. Haghjoo:


It’s a true challenge for treating such young LQTS patient (5-y-o),  
especially, it is difficuilt to increase the dosage of Betablocker  
while patient has low heart rate.  Based our experience ,my  
suggestion is:

1.     To change betablocker from propranolol to Metoprolol extended  
action tablet or nadolol (depending on what you can get in your  
country);

2.     If the patient continues to have syncope episode during  
betablocker administration, recommend her to receive the left  
sympathetic denervation surgery.  We had undergo such surgery for a 6  
y-o boy 5 years ago and for a 7-y-o girl last Sept. in Peking  
University People’s Hospital, China.  It’s not unconquerable for  
surgery though it’s more difficult than that for adult patients. Dr.  
Schwartz’s group has more experiences to deal with patients who need  
surgery.

3.     When her body size is large enough, ICD may be considered  
depending on her symptom at that time.

It’s a wonderful experience to exchange opinions with experts from  
world wide, and thanks to the organizers, thanks to the participants  
from so many countries!

Cuilan Li, Ph. D
Department of Cardiology,
Peking University People’s Hosp.,
Beijing 100044, China
Tel: 86-10-68314422 Ext. 5940
licuilan在gmail.com

--
Dr. Sergio Dubner
President of Scientific Committee

Dr. Edgardo Schapachnik
President of Steering Committee

>
> 1.     3E 患长QT综合症的孩子  Dr. Haghjoo
>
>
> 亲爱的Dubner教授，
>
> 感谢你和你的同事们参加这次的论坛。
>
> 最近，有一个5岁的孩子因为晕厥到我们中心就诊，
> 他没有器质性心脏病的证据。他有先天性的耳聋。有
> 一次晕厥发作时在β受体阻滞剂停药后的48小时内，
> 其余的是在应用普萘洛尔10mg tid的时候。体表心电图
> 发现QTc = 600ms，心率55次/分。我们认为若在儿童期发
> 病，QTc = 600ms对于患者而言就有发生心脏事件较高的
> 危险，并且有植入ICD的指征。然而，瘦小的身体和不
> 合适的治疗产生的远期危险是植入ICD之后必然的不良
> 结果。基线的心率偏慢是β受体阻滞剂加量的限制限
> 制因素。SCD论坛委员会对于我们这个患者有什么建议
> 吗？
>
>
>
> 预先感谢你们的建议。
>
>
> 祝好，
>
>   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
>
>
> 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, confiltered=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
>
>
> --
> Dr. Sergio Dubner
> President of Scientific Committee
>
> Dr. Edgardo Schapachnik
> President of Steering Committee
>
>
>
>
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