[SCD-FORUM] 77E Jervell和Lange-Nielsen综合症和心肌病 Dr. Didier Klug

[SCD Symposium]

77E Jervell和Lange-Nielsen综合症和心肌病  Dr. Didier Klug

我们有一例诊为Lange-Nielsen综合征（KCNQ1）的患儿，尽 
管接受了纳多洛尔的治疗，仍有晕厥和尖端扭转室 
速，后来植入了起搏器，8年内通过右心室起搏和纳 
多洛尔的联合治疗，没有再发尖端扭转室速和晕厥。 
但最近一次的心超提示左室明显扩张，而EF仅18%。我 
们植入了MVP模式的双腔ICD，且心房起搏不造成房室传 
导阻滞。3个月后EF改善（达28%）。您对这个Jerwell心 
肌病有什么看法。对复极明显改变的患者来说，这种 
不同步能否归因于心室起搏或是快速起搏的有害作 
用，或是钙离子的超负荷？


Didier Klug, MD, PhD
Hôpital cardiologique de Lille
University of Lille
France
徐怡琼译 王玲洁校

We follow a young boy with a Jervell and Lange-Nielsen syndrome
(KCNQ1) with syncope and torsade de pointe despite Nadolol requiring
PM implantation at 6 month y.o. Torsade de pointes disappeared after
right ventricular pacing + nadolol without syncope after a 8 years
FU. But last echocardiography has shown a major left ventricular
dilatation with ejection fraction = 18%. We have implanted a dual
chamber ICD with MVP mode and atrial pacing without AV block. The FU
is too short but at 3 months we have the feeling that EF improved
(28%). What do you think about this association Jerwell
cardiomyopathy. Can we incriminate desynchronisation with ventricular
pacing or a deleterious effect of rapid pacing in patient with major
alteration of their repolarization and perhaps calcium overload?

Didier Klug, MD, PhD
Hôpital cardiologique de Lille
University of Lille
France


--
Dr. Sergio Dubner
President of Scientific Committee

Dr. Edgardo Schapachnik
President of Steering Committee