title: Regional and transmural dispersion of repolarisation in patients with Emery-Dreifuss muscular dystrophy.
abstract: BACKGROUND
  The development of malignant ventricular arrhythmias is a possible feature in Emery-Dreifuss muscular dystrophy (EDMD) patients with normal left ventricular systolic function. This event may be the cause of sudden cardiac death in EDMD patients. QTc dispersion (QTc-D), JTc dispersion (JTc-D) and Tpeak-end dispersion (TDR) could reflect the physiological variability of regional and transmural ventricular repolarisation and could provide a substrate for life-threatening ventricular arrhythmias.
  
  
  OBJECTIVE
  The current study was designed to evaluate the heterogeneity of ventricular repolarisation in EDMD patients.
  
  
  METHODS
  Echocardiograms and electrocardiograms from 40 EDMD patients (age 20 ± 13) were evaluated and compared to those of 40 healthy age-matched controls.
  
  
  RESULTS
  The EDMD group, compared to the healthy control group, presented increased values of QTc-D (82.8 ± 44.1 vs. 53.3 ± 13.9, p = 0.003), JTc-D (73.6 ± 32.3 vs. 60.4 ± 11.1 ms, p = 0.001) and TDR (100.54 ± 19.06 vs. 92.15 ± 15.5 ms, p = 0.004). No correlation between QTc dispersion and ejection fraction (R = 0.2, p = 0.3) was found.
  
  
  CONCLUSIONS
  EDMD is associated with significantly increased regional and transmural heterogeneity of ventricular repolarisation, in the absence of impaired systolic and diastolic cardiac function.
authors.affiliation: Department of Cardiorespiratory Sciences, Arrhythmologic Unit, Second University of Naples, Italy.
authors.forename: Gerardo
authors.initials: G
authors.lastname: Nigro
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Russo
authors.forename: Anna
authors.initials: A
authors.lastname: Rago
authors.forename: Andrea Antonio
authors.initials: AA
authors.lastname: Papa
authors.forename: Nicola
authors.initials: N
authors.lastname: Carbone
authors.forename: Michal
authors.initials: M
authors.lastname: Marchel
authors.forename: Alberto
authors.initials: A
authors.lastname: Palladino
authors.forename: Irena
authors.initials: I
authors.lastname: Hausmanowa-Petrusewicz
authors.forename: Maria Giovanna
authors.initials: MG
authors.lastname: Russo
authors.forename: Luisa
authors.initials: L
authors.lastname: Politano
mesh_terms: D000328:Adult; D001145:Arrhythmias, Cardiac / Q000175:diagnosis* / Q000209:etiology*; D001281:Atrial Fibrillation / Q000175:diagnosis; D004452:Echocardiography; D004562:Electrocardiography; D015716:Electrocardiography, Ambulatory; D006327:Heart Block / Q000175:diagnosis; D006801:Humans; D008297:Male; D020389:Muscular Dystrophy, Emery-Dreifuss / Q000150:complications* / Q000175:diagnosis* / Q000503:physiopathology; D012804:Sick Sinus Syndrome / Q000175:diagnosis; D017180:Tachycardia, Ventricular / Q000175:diagnosis; D014693:Ventricular Fibrillation / Q000175:diagnosis