title: Electrocardiographic risk stratification in families with congenital long QT syndrome.
abstract: OBJECTIVE
  The QT interval in the surface ECG is one of the most often used risk stratifiers in families with congenital long QT syndrome (LQTS). The best ECG lead for clinical management of LQTS families remains unclear.
  
  
  RESULTS
  The predictive power of the QTc interval in all ECG leads was studied in 200 consecutive genotyped LQTS family members to identify mutation carriers (n = 103; age: 35+/-19 years) and high-risk LQTS patients (n = 16 with survived sudden cardiac arrest) using receiver operating curve (ROC) analysis (ROC = area under curve). Additionally, the risk for events (syncope and sudden cardiac arrest) was calculated for QTc decile in all individuals. The predictive power was highest in lead II and lead V5 for identifying carriers in LQTS families. These ECG leads were optimal for risk stratification (ROC range 0.83-0.87). In these leads, positive predictive value (PPV) and negative predictive value (NPV) were highest for suggested QTc cut-offs (440 and 500 ms) for identification of LQTS mutation carriers and high-risk patients (PPV between 78-81 and 73-80%, respectively). The risk for events in QTc deciles increased exponentially from 10 to 80% and was 40% for QTc > 500 ms.
  
  
  CONCLUSIONS
  On the basis of these data, QTc is the best diagnostic and prognostic ECG parameter in LQTS families. A single measurement should be obtained in lead II if measurable and then in left precordial leads (preferably V5) as a second choice.
authors.affiliation: Department of Cardiology and Angiology, Medizinische Klinik und Poliklinik C, University Hospital Münster, Albert-Schweitzer-Str. 33, D-48149 Münster, Germany. moennig@uni-muenster.de
authors.forename: Gerold
authors.initials: G
authors.lastname: Mönnig
authors.forename: Lars
authors.initials: L
authors.lastname: Eckardt
authors.forename: Horst
authors.initials: H
authors.lastname: Wedekind
authors.forename: Wilhelm
authors.initials: W
authors.lastname: Haverkamp
authors.forename: Joachim
authors.initials: J
authors.lastname: Gerss
authors.forename: Peter
authors.initials: P
authors.lastname: Milberg
authors.forename: Kristina
authors.initials: K
authors.lastname: Wasmer
authors.forename: Paulus
authors.initials: P
authors.lastname: Kirchhof
authors.forename: Gerd
authors.initials: G
authors.lastname: Assmann
authors.forename: Günter
authors.initials: G
authors.lastname: Breithardt
authors.forename: Eric
authors.initials: E
authors.lastname: Schulze-Bahr
mesh_terms: D000328:Adult; D016757:Death, Sudden, Cardiac / Q000517:prevention & control*; D004562:Electrocardiography / Q000592:standards*; D005260:Female; D006801:Humans; D008133:Long QT Syndrome / Q000151:congenital* / Q000175:diagnosis / Q000235:genetics; D008297:Male; D021141:Nucleic Acid Amplification Techniques; D010375:Pedigree; D012372:ROC Curve; D018570:Risk Assessment / Q000379:methods / Q000592:standards; D012680:Sensitivity and Specificity