[SCD-FORUM] 28E 患有长QT综合征（LQTS）的年轻女性 Kukla医生

[SCD Symposium]

28E 患有长QT综合征（LQTS）的年轻女性  Kukla医生

     首先，感谢此次心脏猝死在线论坛。
     我想请教一下世界著名的LQTS专家Silvia Priori教 
授。我的一名23岁女性患者，2年前，即第一次发作 
（也是唯一一次）后的五个月被诊断为LQTS.。她当时 
意识丧失。静息ECG上QT间期为515ms，Holter室早为 
300/24h，夜间QT高达600ms，T波交替，期外收缩TU波增 
宽。运动试验可诱发单形室早（单形性二联律），并 
在恢复期完全消失。静息心电图显示V2-V5导联T波有切 
迹，所以它似像2型LQTS.她没有心脏猝死家族史。我们 
开始给她加用β受体阻滞剂——美托洛尔直到剂量达 
3×75mg（该剂量患者能很好耐受，不再出现晕厥）。 
（Nadolol波兰目前没有）。因为她打算再一次怀孕， 
所以我的问题是：
1）妊娠期当如何正确治疗？
2）如何使患者平安度过分娩期？
3）产后是否应该增加β受体阻滞剂的剂量？

  致

  Piotr Kukla医生
  Gorlice专科医院
  内科
  Gorlice, 波兰

Sergio Dubner博士
  科委会主席

Edgardo Schapachnik博士
组委会主席

28E Young woman with LQTS. Dr. Kukla
  First of all I would like to say "thank you" for the nice on-line   
Symposium on SCD.
I would kindly ask Prof. Silvia Priori....worldwide famous expert on   
LQTS；Namely, the one of my patient is a 23-yo woman.....LQTS was  
diagnosed 2 years ago. It was 5 months after first (and the only)  
partum. She had  lost of conscious. QT interval in rest ECG - 515  
msec, in Holter monitorng....PVc -300/day, QT in nighttime till 600  
msec, dynamicity in T-wave complex,postextrasystolic TU wave  
augmentation. Exercise test induced  momomorphic PVC (monomorphic  
bigeminy)......PVC disapeared totally in recovery  phase.

Rest ECG shows notched T wave in V2-V5 so it looks like LQTS type  
2.There is no evidence of SCD in her family. We started treatment  
with Beta-bloker -metoprolol until dose  3 x 75 mg (this dose is  
well  Tolerated by her...with no syncope so far). (Nadolol is not  
available in Poland  now).Because she plans to have the next  
pregnancy. My questions are.
1) what should be the proper therapy during pregnancy ?

2) how to carry safe the patient during the delivery period ?

3) should I increase the dose of beta-bloker in post-partum period ?

with best regard,

Dr Piotr Kukla
Specialistic Hospital in Gorlice
Departament of Internal Medicine
Gorlice, Poland


--
Dr. Sergio Dubner
President of Scientific Committee

Dr. Edgardo Schapachnik
President of Steering Committee