title: Increased dispersion of ventricular repolarization in Emery Dreifuss muscular dystrophy patients.
abstract: BACKGROUND
  Sudden cardiac death (SCD) is common in patients with Emery-Dreifuss muscular dystrophy (EDMD) and is attributed to the development of life-threatening arrhythmias that occur in the presence of normal left ventricular systolic function. Heterogeneity of ventricular repolarization is considered to provide an electrophysiological substrate for malignant arrhythmias. QTc dispersion (QTc-D) and JTc dispersion (JTc-D) are electrocardiographic parameters indicative of heterogeneity of ventricular repolarization. The aim of our study was to evaluate the heterogeneity of ventricular repolarization in patients with Emery-Dreifuss muscular dystrophy with preserved systolic and diastolic cardiac function.
  
  
  METHODS
  The study involved 36 EDMD patients (age 20 ± 12, 26 M) and 36 healthy subjects used as controls, matched for age and sex. Heart rate, QRS duration, maximum and minimum QT and JT interval, QTc-D and JTc-D measurements were performed.
  
  
  RESULTS
  Compared to the healthy control group, the EDMD group presented increased values of QTc-D (82.7 ± 44.2 vs. 53.1 ± 13.7; P=0,003) and JTc-D (73.6 ± 32.3 vs. 60.4 ± 11.1 ms; P=0.001). No correlation between QTc dispersion and ejection fraction (R=0.2, P=0.3) was found.
  
  
  CONCLUSIONS
  Our study showed a significant increase of QTc-D and JTc-D in Emery-Dreifuss muscular dystrophy patients with preserved systolic and diastolic cardiac function.
authors.affiliation: Chair of Cardiology, 2nd University of Naples, Naples, Italy.
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Russo
authors.forename: Anna
authors.initials: A
authors.lastname: Rago
authors.forename: Luisa
authors.initials: L
authors.lastname: Politano
authors.forename: Andrea Antonio
authors.initials: AA
authors.lastname: Papa
authors.forename: Federica
authors.initials: F
authors.lastname: Di Meo
authors.forename: Maria Giovanna
authors.initials: MG
authors.lastname: Russo
authors.forename: Paolo
authors.initials: P
authors.lastname: Golino
authors.forename: Raffaele
authors.initials: R
authors.lastname: Calabrò
authors.forename: Gerardo
authors.initials: G
authors.lastname: Nigro
mesh_terms: D016022:Case-Control Studies; D004562:Electrocardiography; D005260:Female; D006352:Heart Ventricles / Q000503:physiopathology*; D006801:Humans; D008297:Male; D020389:Muscular Dystrophy, Emery-Dreifuss / Q000000981:diagnostic imaging / Q000503:physiopathology*; D014463:Ultrasonography; D016276:Ventricular Function / Q000502:physiology*; D055815:Young Adult