[SCD-FORUM] 3E 患长QT综合症的孩子 Dr. Haghjoo

[SCD Symposium]

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