open access

Vol 70, No 11 (2012)
Original articles
Published online: 2012-11-21
Submitted: 2012-12-28
Get Citation

Regional and transmural dispersion of repolarisation in patients with Emery−Dreifuss muscular dystrophy

Gerardo Nigro, Vincenzo Russo, Anna Rago, Andrea Antonio Papa, Nicola Carboni, Michal Marchel, Alberto Palladino, Irena Hausmanowa−Petrusewicz, Maria Giovanna Russo, Luisa Politano
Kardiol Pol 2012;70(11):1154-1159.

open access

Vol 70, No 11 (2012)
Original articles
Published online: 2012-11-21
Submitted: 2012-12-28

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.
Aim:
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.

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.
Aim:
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.
Get Citation

Keywords

Emery-Dreifuss muscular dystrophy (EDMD); sudden cardiac death; ventricular repolarisation; QTc dispersion; JTc dispersion; Tpeak-end dispersion; transmural dispersion of repolarisation

About this article
Title

Regional and transmural dispersion of repolarisation in patients with Emery−Dreifuss muscular dystrophy

Journal

Kardiologia Polska (Polish Heart Journal)

Issue

Vol 70, No 11 (2012)

Pages

1154-1159

Published online

2012-11-21

Bibliographic record

Kardiol Pol 2012;70(11):1154-1159.

Keywords

Emery-Dreifuss muscular dystrophy (EDMD)
sudden cardiac death
ventricular repolarisation
QTc dispersion
JTc dispersion
Tpeak-end dispersion
transmural dispersion of repolarisation

Authors

Gerardo Nigro
Vincenzo Russo
Anna Rago
Andrea Antonio Papa
Nicola Carboni
Michal Marchel
Alberto Palladino
Irena Hausmanowa−Petrusewicz
Maria Giovanna Russo
Luisa Politano

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.

By "Via Medica sp. z o.o." sp.k., Świętokrzyska 73 street, 80–180 Gdańsk, Poland

tel.:+48 58 320 94 94, faks:+48 58 320 94 60, e-mail: viamedica@viamedica.pl