[SCD-FORUM] 77E Jervell and Lange-Nielsen syndrome and cardiomyopathy. Dr. Didier Klug

[SCD Symposium]

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




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