Increased heterogeneity of ventricular repolarization in myotonic dystrophy type 1 population.
Sudden cardiac death in myotonic dystrophy type I (DM1) patients can be attributed to atrioventricular blocks as far as to the development of life-threatening arrhythmias which occur even in hearts with normal left ventricular systolic and diastolic function. Heterogeneity of ventricular repolarization is considered to provide an electrophysiological substrate for malignant arrhythmias. QTc dispersion (QTc-D), JTc dispersion (JTc-D) and transmural dispersion of repolarization (TDR) could reflect the physiological variability of regional and transmural ventricular repolarization. Aim of the present study was to investigate the heterogeneity of ventricular repolarization in patients with DM1 and preserved diastolic and systolic cardiac function. The study enrolled 50 DM1 patients (mean age 44 ± 5 years; M:F: 29:21) with preserved systolic and diastolic function of left ventricle among 247 DM1 patients followed at Cardiomyology and Medical Genetics of Second University of Naples, and 50 sexand age-matched healthy controls. The electrocardiographic parameters investigated were the following: Heart Rate, QRS duration, maximum and minimum QT and JT intervals, QTc- D, JTc-D and TDR. Compared to the controls, the DM1 group presented increased values of QTc-D (86.7 ± 40.1 vs 52.3 ± 11.9 ms; p = 0.03), JTc-D (78.6 ± 31.3 vs 61.3 ± 10.2 ms; p = 0.001) and TDR (101.6 ± 18.06 vs 90.1 ± 14.3 ms; p = 0.004) suggesting a significant increase in regional and transmural heterogeneity of the ventricular repolarization in these patients, despite a preserved systolic and diastolic cardiac function.
Cardiomyology and Medical Genetics, Department of Experimental Medicine, Second University of Naples and Monaldi Hospital, Naples, Italy.
Vincenzo
V
Russo
Cardiomyology and Medical Genetics, Department of Experimental Medicine, Second University of Naples and Monaldi Hospital, Naples, Italy.
Andrea Antonio
AA
Papa
Cardiomyology and Medical Genetics, Department of Experimental Medicine, Second University of Naples and Monaldi Hospital, Naples, Italy.
Anna
A
Rago
Cardiomyology and Medical Genetics, Department of Experimental Medicine, Second University of Naples and Monaldi Hospital, Naples, Italy.
Paola
P
D'Ambrosio
Cardiomyology and Medical Genetics, Department of Experimental Medicine, Second University of Naples and Monaldi Hospital, Naples, Italy.
Giovanni
G
Cimmino
Cardiomyology and Medical Genetics, Department of Experimental Medicine, Second University of Naples and Monaldi Hospital, Naples, Italy.
Alberto
A
Palladino
Cardiomyology and Medical Genetics, Department of Experimental Medicine, Second University of Naples and Monaldi Hospital, Naples, Italy.
Luisa
L
Politano
Cardiomyology and Medical Genetics, Department of Experimental Medicine, Second University of Naples and Monaldi Hospital, Naples, Italy.
Gerardo
G
Nigro
D000328:Adult; D001145:Arrhythmias, Cardiac / Q000209:etiology / Q000503:physiopathology*; D016022:Case-Control Studies; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000150:complications / Q000503:physiopathology*; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology*
JTc dispersion; QTc dispersion; arrhythmias; myotonic dystrophy; repolarization; sudden cardiac death