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Increased dispersion of ventricular repolarization in Emery Dreifuss muscular dystrophy patients

Vincenzo Russo1, Anna Rago, Luisa Politano

  • 1Chair of Cardiology, 2nd University of Naples, Naples, Italy.

Medical Science Monitor : International Medical Journal of Experimental and Clinical Research
|November 1, 2012
PubMed

Insights

Sudden cardiac death in Emery-Dreifuss muscular dystrophy (EDMD) patients is linked to arrhythmias. This study found increased QTc dispersion (QTc-D) and JTc dispersion (JTc-D) in EDMD patients, indicating heightened risk.

Area of Science:

  • Cardiology
  • Genetics
  • Neurology

Background:

  • Sudden cardiac death (SCD) is a significant concern in Emery-Dreifuss muscular dystrophy (EDMD).
  • Life-threatening arrhythmias in EDMD occur despite normal left ventricular systolic function.
  • Ventricular repolarization heterogeneity, indicated by QTc dispersion (QTc-D) and JTc dispersion (JTc-D), is a potential substrate for these arrhythmias.

Purpose of the Study:

  • To assess ventricular repolarization heterogeneity in EDMD patients.
  • To evaluate QTc-D and JTc-D in EDMD patients with preserved cardiac function.

Main Methods:

  • The study included 36 EDMD patients and 36 healthy controls, matched for age and sex.
  • Electrocardiographic parameters including QTc-D and JTc-D were measured.
  • Analysis compared EDMD patients to the control group.

Main Results:

  • EDMD patients exhibited significantly higher QTc-D (82.7 ± 44.2 ms) compared to controls (53.1 ± 13.7 ms; P=0.003).
  • EDMD patients also showed significantly higher JTc-D (73.6 ± 32.3 ms) versus controls (60.4 ± 11.1 ms; P=0.001).
  • No correlation was found between QTc dispersion and ejection fraction (R=0.2, P=0.3).

Conclusions:

  • Emery-Dreifuss muscular dystrophy patients with preserved cardiac function demonstrate increased QTc-D and JTc-D.
  • These findings suggest heightened ventricular repolarization heterogeneity in EDMD.
  • This heterogeneity may contribute to the arrhythmogenic risk in EDMD patients.
Abstract

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