title: The Prevalence and Significance of the Early Repolarization Pattern in Sudden Arrhythmic Death Syndrome Families.
abstract: The early repolarization (ER) pattern is associated with sudden death and has been shown to be heritable. Its significance when identified in families affected by sudden arrhythmic death syndrome (SADS) remains unclear.
  
  
  
  We analyzed 12-lead ECGs of 401 first-degree relatives of individuals who had died from SADS. The prevalence of ER patterns was compared with family-clustered controls. ER was more common in SADS family members than in controls (21% versus 8%; odds ratio: 5.14; 95% confidence interval, 3.37-7.84) independent of the presence of a familial cardiac diagnosis. Both ascending and horizontal ER patterns were more common. In addition, ER was investigated for associations with findings from ajmaline provocation (n=332), exercise ECG (n=304), and signal-averaged ECG (n=118) when performed. ER was associated with a trend toward late depolarization, in general was suppressed with exercise and was unaffected by ajmaline. Inferior and horizontal patterns were, however, more likely to persist during exercise. Augmentation of ER with ajmaline was rare.
  
  
  
  The ER pattern is more common in SADS family members than controls adjusted in particular for relatedness. The increased prevalence is irrespective of ER subtype and the presence of other inherited arrhythmia syndromes. ER may therefore represent an underlying heritable arrhythmia syndrome or risk factor for sudden death in the context of other cardiac pathology. The differing response of ER subtypes to exercise and ajmaline provocation suggests underlying mechanisms of both abnormal repolarization and depolarization.
authors.affiliation: From the Cardiac Research Centre, St. George's University of London, London, United Kingdom (G.M., C.R., H.R., Y.W., M.P., S.S., E.R.B.); Department of Cardiovascular Sciences, University of Leicester & National Institute for Health Research Leicester Cardiovascular Biomedical Research Unit, Leicester, United Kingdom (C.P.N., N.J.S.); Deutsches Herzzentrum München, Klinik für Herz- und Kreislauferkrankungen, Technische Universität München, München, Germany (C.H., W.R.); and Deutsches Zentrum für Herz- und Kreislauf-Forschung (DZHK) e.V. (German Center for Cardiovascular Research), Partner Site Munich Heart Alliance, Munich, Germany (C.H.).
authors.forename: Greg
authors.initials: G
authors.lastname: Mellor
authors.affiliation: From the Cardiac Research Centre, St. George's University of London, London, United Kingdom (G.M., C.R., H.R., Y.W., M.P., S.S., E.R.B.); Department of Cardiovascular Sciences, University of Leicester & National Institute for Health Research Leicester Cardiovascular Biomedical Research Unit, Leicester, United Kingdom (C.P.N., N.J.S.); Deutsches Herzzentrum München, Klinik für Herz- und Kreislauferkrankungen, Technische Universität München, München, Germany (C.H., W.R.); and Deutsches Zentrum für Herz- und Kreislauf-Forschung (DZHK) e.V. (German Center for Cardiovascular Research), Partner Site Munich Heart Alliance, Munich, Germany (C.H.).
authors.forename: Christopher P
authors.initials: CP
authors.lastname: Nelson
authors.affiliation: From the Cardiac Research Centre, St. George's University of London, London, United Kingdom (G.M., C.R., H.R., Y.W., M.P., S.S., E.R.B.); Department of Cardiovascular Sciences, University of Leicester & National Institute for Health Research Leicester Cardiovascular Biomedical Research Unit, Leicester, United Kingdom (C.P.N., N.J.S.); Deutsches Herzzentrum München, Klinik für Herz- und Kreislauferkrankungen, Technische Universität München, München, Germany (C.H., W.R.); and Deutsches Zentrum für Herz- und Kreislauf-Forschung (DZHK) e.V. (German Center for Cardiovascular Research), Partner Site Munich Heart Alliance, Munich, Germany (C.H.).
authors.forename: Claire
authors.initials: C
authors.lastname: Robb
authors.affiliation: From the Cardiac Research Centre, St. George's University of London, London, United Kingdom (G.M., C.R., H.R., Y.W., M.P., S.S., E.R.B.); Department of Cardiovascular Sciences, University of Leicester & National Institute for Health Research Leicester Cardiovascular Biomedical Research Unit, Leicester, United Kingdom (C.P.N., N.J.S.); Deutsches Herzzentrum München, Klinik für Herz- und Kreislauferkrankungen, Technische Universität München, München, Germany (C.H., W.R.); and Deutsches Zentrum für Herz- und Kreislauf-Forschung (DZHK) e.V. (German Center for Cardiovascular Research), Partner Site Munich Heart Alliance, Munich, Germany (C.H.).
authors.forename: Hariharan
authors.initials: H
authors.lastname: Raju
authors.affiliation: From the Cardiac Research Centre, St. George's University of London, London, United Kingdom (G.M., C.R., H.R., Y.W., M.P., S.S., E.R.B.); Department of Cardiovascular Sciences, University of Leicester & National Institute for Health Research Leicester Cardiovascular Biomedical Research Unit, Leicester, United Kingdom (C.P.N., N.J.S.); Deutsches Herzzentrum München, Klinik für Herz- und Kreislauferkrankungen, Technische Universität München, München, Germany (C.H., W.R.); and Deutsches Zentrum für Herz- und Kreislauf-Forschung (DZHK) e.V. (German Center for Cardiovascular Research), Partner Site Munich Heart Alliance, Munich, Germany (C.H.).
authors.forename: Yanushi
authors.initials: Y
authors.lastname: Wijeyeratne
authors.affiliation: From the Cardiac Research Centre, St. George's University of London, London, United Kingdom (G.M., C.R., H.R., Y.W., M.P., S.S., E.R.B.); Department of Cardiovascular Sciences, University of Leicester & National Institute for Health Research Leicester Cardiovascular Biomedical Research Unit, Leicester, United Kingdom (C.P.N., N.J.S.); Deutsches Herzzentrum München, Klinik für Herz- und Kreislauferkrankungen, Technische Universität München, München, Germany (C.H., W.R.); and Deutsches Zentrum für Herz- und Kreislauf-Forschung (DZHK) e.V. (German Center for Cardiovascular Research), Partner Site Munich Heart Alliance, Munich, Germany (C.H.).
authors.forename: Christian
authors.initials: C
authors.lastname: Hengstenberg
authors.affiliation: From the Cardiac Research Centre, St. George's University of London, London, United Kingdom (G.M., C.R., H.R., Y.W., M.P., S.S., E.R.B.); Department of Cardiovascular Sciences, University of Leicester & National Institute for Health Research Leicester Cardiovascular Biomedical Research Unit, Leicester, United Kingdom (C.P.N., N.J.S.); Deutsches Herzzentrum München, Klinik für Herz- und Kreislauferkrankungen, Technische Universität München, München, Germany (C.H., W.R.); and Deutsches Zentrum für Herz- und Kreislauf-Forschung (DZHK) e.V. (German Center for Cardiovascular Research), Partner Site Munich Heart Alliance, Munich, Germany (C.H.).
authors.forename: Wibke
authors.initials: W
authors.lastname: Reinhard
authors.affiliation: From the Cardiac Research Centre, St. George's University of London, London, United Kingdom (G.M., C.R., H.R., Y.W., M.P., S.S., E.R.B.); Department of Cardiovascular Sciences, University of Leicester & National Institute for Health Research Leicester Cardiovascular Biomedical Research Unit, Leicester, United Kingdom (C.P.N., N.J.S.); Deutsches Herzzentrum München, Klinik für Herz- und Kreislauferkrankungen, Technische Universität München, München, Germany (C.H., W.R.); and Deutsches Zentrum für Herz- und Kreislauf-Forschung (DZHK) e.V. (German Center for Cardiovascular Research), Partner Site Munich Heart Alliance, Munich, Germany (C.H.).
authors.forename: Michael
authors.initials: M
authors.lastname: Papadakis
authors.affiliation: From the Cardiac Research Centre, St. George's University of London, London, United Kingdom (G.M., C.R., H.R., Y.W., M.P., S.S., E.R.B.); Department of Cardiovascular Sciences, University of Leicester & National Institute for Health Research Leicester Cardiovascular Biomedical Research Unit, Leicester, United Kingdom (C.P.N., N.J.S.); Deutsches Herzzentrum München, Klinik für Herz- und Kreislauferkrankungen, Technische Universität München, München, Germany (C.H., W.R.); and Deutsches Zentrum für Herz- und Kreislauf-Forschung (DZHK) e.V. (German Center for Cardiovascular Research), Partner Site Munich Heart Alliance, Munich, Germany (C.H.).
authors.forename: Sanjay
authors.initials: S
authors.lastname: Sharma
authors.affiliation: From the Cardiac Research Centre, St. George's University of London, London, United Kingdom (G.M., C.R., H.R., Y.W., M.P., S.S., E.R.B.); Department of Cardiovascular Sciences, University of Leicester & National Institute for Health Research Leicester Cardiovascular Biomedical Research Unit, Leicester, United Kingdom (C.P.N., N.J.S.); Deutsches Herzzentrum München, Klinik für Herz- und Kreislauferkrankungen, Technische Universität München, München, Germany (C.H., W.R.); and Deutsches Zentrum für Herz- und Kreislauf-Forschung (DZHK) e.V. (German Center for Cardiovascular Research), Partner Site Munich Heart Alliance, Munich, Germany (C.H.).
authors.forename: Nilesh J
authors.initials: NJ
authors.lastname: Samani
authors.affiliation: From the Cardiac Research Centre, St. George's University of London, London, United Kingdom (G.M., C.R., H.R., Y.W., M.P., S.S., E.R.B.); Department of Cardiovascular Sciences, University of Leicester & National Institute for Health Research Leicester Cardiovascular Biomedical Research Unit, Leicester, United Kingdom (C.P.N., N.J.S.); Deutsches Herzzentrum München, Klinik für Herz- und Kreislauferkrankungen, Technische Universität München, München, Germany (C.H., W.R.); and Deutsches Zentrum für Herz- und Kreislauf-Forschung (DZHK) e.V. (German Center for Cardiovascular Research), Partner Site Munich Heart Alliance, Munich, Germany (C.H.). ebehr@sgul.ac.uk.
authors.forename: Elijah R
authors.initials: ER
authors.lastname: Behr
mesh_terms: D000328:Adult; D000404:Ajmaline; D001145:Arrhythmias, Cardiac / Q000235:genetics* / Q000503:physiopathology*; D016022:Case-Control Studies; D003645:Death, Sudden; D016757:Death, Sudden, Cardiac; D004562:Electrocardiography; D005260:Female; D006801:Humans; D008297:Male; D015995:Prevalence; D012307:Risk Factors
chemical_list: D000404:Ajmaline
keywords: ajmaline; death, sudden; electrocardiography