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P-Wave Duration and Dispersion in Patients With Emery-Dreifuss Muscular Dystrophy
  1. Vincenzo Russo, MD*,
  2. Anna Rago, MD*,
  3. Alberto Palladino, MD,
  4. Andrea Antonio Papa, MD*,
  5. Federica Di Meo, MD*,
  6. Nadia Della Cioppa, MD,
  7. Paolo Golino, MD, PhD*,
  8. Maria Giovanna Russo, MD*,
  9. Raffaele Calabrò, MD*,
  10. Luisa Politano, MD,
  11. Gerardo Nigro, MD, PhD*
  1. From the *Department of Cardiology, Second University of Naples-Monaldi Hospital; †Cardiomyology and Genetic Section, Department of Internal and Experimental Medicine, Second University of Naples; and ‡Department of Anestesy and Rianimation, Monaldi Hospital, Naples, Italy.
  1. Received March 31, 2011, and in revised form June 10, 2011.
  2. Accepted for publication June 11, 2011.
  3. Reprints: Vincenzo Russo, MD, Via della Resistenza, 48, 80021 Afragola, Naples, Italy. E-mail: v.p.russo{at}


Background Paroxysmal episodes of atrial fibrillation frequently occur in Emery-Dreifuss muscular dystrophy (EDMD). Although previous studies have documented a variety of electrocardiographic abnormalities in EDMD, little is still known about P-wave dispersion (PD), an independent risk factor for the development of atrial fibrillation. The aim of our study was to evaluate the P-wave duration and PD in patients with EDMD with conserved systolic and diastolic cardiac function.

Methods The study involved 36 patients with EDMD (age, 20 [SD, 12] years; 26 men) and 36 healthy subjects used as controls, matched for age and sex. P-wave dispersion was carefully measured using 12-lead electrocardiogram. Compared with the healthy control group, patients with EDMD presented increased maximum P-wave duration (108.2 [SD, 22.2] vs 97.8 [SD, 11] milliseconds, P = 0.04) and PD (51.4 [SD, 12.8] vs 39.3 [SD, 9.7] milliseconds, P = 0.004) values. No statistically significant differences in left atrium diameter (37.1 [SD, 2.9] vs 34.1 [SD, 4.2] mm, P = 0.3) and maximum left atrium volume (15.2 [SD, 3.8] vs 14.1 [SD, 4.2] mL/m2, P = 0.4) were found between the 2 groups. We divided our study population into 2 subgroups, according to the different genetic diagnosis, patients with laminopathy EDMD (n = 17) or with emerinopathy EDMD (n = 19). No statistically significant differences were found in PD between the 2 subgroups (54.6 [SD, 15.6] vs 50.2 [SD, 11.5] milliseconds, P = 0.4).

Conclusions Our study showed a significant increase of maximum P-wave duration and PD in patients with EDMD with conserved systolic and diastolic cardiac function.

Key Words
  • Emery-Dreifuss muscular dystrophy
  • P-wave dispersion
  • atrial fibrillation
  • arrhythmias

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