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Ventricular Late Potentials in Myotonic Dystrophy
Ann Int Med 115:607-613, Milner,M.R.,et al, 1991
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Article Abstract
A time-domain analysis of the signal-averaged electrocardiograms showed that 75%of patients with myotonic dystrophy met one criterion for the presence of late potentials,67%met two criteria,and 29%met all three criteria.Spectrotemporal mapping in these patients showed markedly abnormal spectral peaks with a mean factor of normality that was significantly lower than that of the normal volunteers;the frequency of electrocardiographic abnormalities approached that seen in patients with known ventricular tachycardia.The presence of late potentials correlated directly with the length of the PR interval and inversely with left ventricular fractional shortening.In our study,the prevalence of late potentials on signal-averaged electrocadiography in patients with myotonic dystrophy approached that seen in cardiac patients with inducible ventricular tachycardia.It is possible that ventricular arrhythmias play a role in the occurrence of sudden death in some patients with myotonic dystrophy.
 
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arrhythmia,cardiac
electrocardiogram,abnormal
muscular dystrophy
muscular dystrophy,cardiovascular changes with
myotonia dystrophica
sudden death
ventricular tachycardia

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