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European Heart Journal 1993 14(5):669-676;
Copyright © 1993 by the European Society of Cardiology.
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© 1993 The Europen Society of Cardiology

Discrepancy between systolic and diastolic dysfunction of the left ventricle in patients with Duchenne muscular dystrophy

A. TAKENAKA, M. YOKOTA*,, M. IWASE, K. MIYAGUCHI, H. HAYASHI and H. SAITO

First Department of Internal Medicine, Nagoya University School of Medicine Nagoya, Japan
*Department of Clinical Laboratory, Nagoya University Hospital Nagoya, Japan

Received 26 May 1992; revised 19 October 1992; .

Correspondence Mitsuhiro Yokota, MD, Department of Clinical Laboratory, Nagoya University Hospital, 65, Tsurumai-cho, Showa-ku, Nagoya, Japan, 466

Abstract

To assess the systolic and diastolic dysfunction of the left ventricle (LV) in relation to age and the severity of impairment in Duchenne muscular dystrophy (DMD), we performed M-mode, two-dimensional and pulsed-wave Doppler echocardi-ography in 45 male subjects with DMD aged 8 to 25 years and in 40 age-matched healthy controls. Systolic dysfunction started in the first decade of life, with some patients showing severe systolic dysfunction in their early teens. This dysfunction, however, did not always depend on the severity of the skeletal muscle disease. No patients with DMD showed an increase in peak atrial velocity and time-velocity integrals of the atrial contraction velocity curve, findings frequently reported to precede the abnormalities in many cardiac diseases; it was thought therefore that these patients had no increase in left atrial compensation. Diastolic dysfunction may not routinely precede or accompany the systolic dysfunction in DMD, in contrast with what is reported in patients with ischaemic or hypertensive heart disease. DMD patients usually show a predominant systolic dysfunction.

Key Words: Duchenne muscular dystrophy • M-mode echocardiography • two-dimensional echocardiography • pulsed-wave echocardiography • systolic dysfunction • diastolic dysfunction


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