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Ventricular Late Potentials in Myotonic Dystrophy

Mark R. Milner, MD; Rollin J. Hawley, MD; Mark Jachim; Joseph Lindsay Jr., MD; and Ross D. Fletcher, MD
[+] Article, Author, and Disclosure Information

This paper was presented in part at the 39th Annual Scientific Session of the American College of Cardiology, March 1990.

Requests for Reprints: Mark Milner, MD, 6410 Rockledge Drive, Suite 200, Bethesda, MD 20817.

Current Author Addresses: Dr. Milner: 6410 Rockledge Drive, Suite 200, Bethesda, MD 20817.

Drs. Hawley and Fletcher: Veterans Affairs Hospital, 50 Irving Street NW, Washington, DC 20010.

Dr. Lindsay: Washington Hospital Center, 110 Irving Street NW, Washington, DC 20010.

Mr. Jachim: Deceased.

From the Veterans Affairs Medical Center, Georgetown University Medical School, Washington Hospital Center, and the George Washington School of Medicine, Washington, DC. For current author addresses, see end of text.†Deceased.

Ann Intern Med. 1991;115(8):607-613. doi:10.7326/0003-4819-115-8-607
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Objective: To determine the prevalence of ventricular late potentials, as determined by signal-averaged electrocardiography, in patients with myotonic dystrophy.

Design: Cross sectional, with blinded analysis of all electrocardiographic data.

Setting: Outpatient departments of a Veterans Affairs medical center and a tertiary care private hospital.

Participants: Twenty-four patients with myotonic dystrophy. Patients were excluded from the study if they had either a history suggestive of significant ventricular arrhythmias or electrocardiographic evidence of a bundle-branch block. Two comparison groups were also formed; one group included 44 healthy employees at the tertiary hospital and the other, 30 cardiac patients with inducible ventricular tachycardia.

Main Results: 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.

▪Conclusions: In our study, the prevalence of late potentials on signal-averaged electrocardiography 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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