title: [Long QT-syndromes: diagnosis and genetics].
abstract: The long-QT syndrome (LQTS) is a familiar disease characterized by abnormal myocardial repolarization and a high risk of sudden cardiac death. As a hallmark of the disease, the heart-rate corrected QT interval is intrinsically prolonged. Recent advances in molecular genetics have elicited that various inborn defects in cardiac ion channel genes regulating cardiac ion currents underlie this propensity to develop malignant ventricular arrhythmias. Meanwhile, a widespread locus and allelic genetic heterogeneity in LQTS is evident, thus, complicating the power of DNA diagnostic tools. The following review will briefly summarize clinical and genetic aspects of LQTS.
authors.affiliation: AG "Genetics of Arrhythmias" Molekular-Kardiologie Institut für Arterioskleroseforschung an der Westfälischen Wilhelms-Universität (WWU) Münster Domagkstr. 3 48149 Münster, Germany Tel.: ++49-251-8352982 Fax: ++49-251-83-52980 E-Mail: heart@uni-muenster.de, DE.
authors.forename: Eric
authors.initials: E
authors.lastname: Schulze-Bahr
authors.forename: Gerold
authors.initials: G
authors.lastname: Mönnig
authors.forename: Dörte
authors.initials: D
authors.lastname: Etzrodt
authors.forename: Hartmut
authors.initials: H
authors.lastname: Fenge
authors.forename: Harald
authors.initials: H
authors.lastname: Funke
authors.forename: Petra
authors.initials: P
authors.lastname: Seiler
authors.forename: Horst
authors.initials: H
authors.lastname: Wedekind
authors.forename: Günter
authors.initials: G
authors.lastname: Breithardt
authors.forename: Wilhelm
authors.initials: W
authors.lastname: Haverkamp
vernacular_title: Angeborene QT-Syndrome : Diagnostik und Genetik.