[SCD-FORUM] 22E RE: 一例长QT综合征患儿一 Dubner 博士

[SCD Symposium]

亲爱的Majid Haghoo 医师:
你描述了一种处理棘手的情况，我不是儿科专家。这 
个患儿出现超过500ms的长QTc而且伴有先天性耳聋，肯 
定属于高危组。如果这个孩子年龄大一点的话，应该 
植入ICD。而他只有5岁，植入ICD带来的问题可能超过 
所获得的利益。

在这类病人中，植入和维护ICD存在许多问题。在特殊 
的情况下可能需运用一些不同的植入技术。这位患儿 
在药物治疗的基础上再次发生长QT的症状可作为植入 
指征。必要时，根据世界各专家的意见，我可能会在 
腹部放置电池盒，在后背经皮下埋置电极。此外，我 
认为对此患儿进行基因测定是很重要的。分析基因突 
变能够指导药物治疗。你应该联系纽约罗彻斯特大学 
的Arthur Moss博士他会将你的病人登记入组并进行测 
定。南卡罗莱纳州医科大学的Philip Saul博士可提供手 
术技能。


致
Sergio Dubner博士
-——
Sergio Dubner博士
科委会主席
Edgardo Schapachnik 博士
组委会主席



22E RE: A children with LQTS. Dr. Dubner

Dear Dr. Majid HaghooYou 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