[Long QT syndrome--genes, mechanisms and risks. Indication for genetic family screening?].
Inherited long QT syndrome (LQTS) is a cardiac disease characterised by episodes of ventricular tachyarrhythmia, presenting as syncope or sudden death. Untreated, the annual mortality rate is 1-2%. Sudden death has been reported as the first manifestation of the disease in some cases. Therefore, early (pre-symptomatic) diagnosis and management may save lives. However, clinically false negative relatives are also at risk of sudden death. On this basis we conclude assessment of relatives should be extended with genetic testing.
Bundgaard
Henning
H
H:S Rigshospitalet, Hjertecentret, Statens Serum Institut, Klinisk Biokemisk Afdeling, og Arhus Universitetshospital, Skejby Sygehus, Hjertemedicinsk Afdeling. henningbundgaard@dadlnet.dk
Havndrup
Ole
O
Christiansen
Michael
M
Andersen
Paal Skytt
PS
Jensen
Henrik Kjaerulff
HK
Svendsen
Jesper Hastrup
JH
Kjeldsen
Keld P
KP
D016757:Death, Sudden, Cardiac / Q000209:etiology / Q000517:prevention & control; D020022:Genetic Predisposition to Disease; D005820:Genetic Testing; D006801:Humans; D007473:Ion Channels / Q000502:physiology; D008133:Long QT Syndrome / Q000150:complications / Q000175:diagnosis / Q000235:genetics*; D012307:Risk Factors; D013575:Syncope / Q000175:diagnosis / Q000235:genetics
D007473:Ion Channels