P-wave duration and dispersion in patients with Emery-Dreifuss muscular dystrophy.
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.
Department of Cardiology, Second University of Naples-Monaldi Hospital, Naples, Italy. v.p.russo@libero.it
Vincenzo
V
Russo
Anna
A
Rago
Alberto
A
Palladino
Andrea Antonio
AA
Papa
Federica
F
Di Meo
Nadia
N
Della Cioppa
Paolo
P
Golino
Maria Giovanna
MG
Russo
Raffaele
R
Calabrò
Luisa
L
Politano
Gerardo
G
Nigro
D000328:Adult; D001281:Atrial Fibrillation / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D015992:Body Mass Index; D016022:Case-Control Studies; D003971:Diastole; D004562:Electrocardiography / Q000379:methods*; D005260:Female; D005819:Genetic Markers; D006325:Heart Atria / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D020389:Muscular Dystrophy, Emery-Dreifuss / Q000150:complications / Q000503:physiopathology*; D012306:Risk; D012307:Risk Factors; D013599:Systole; D013997:Time Factors
D005819:Genetic Markers