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Heart 1998;80:292-295; doi:10.1136/hrt.80.3.292
Copyright © 1998 BMJ Publishing Group Ltd & British Cardiovascular Society

Heart 1998;80:292-295 ( September )

A follow up study of myocardial involvement in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS)

Y Okajima,a Y Tanabe,b M Takayanagi,c H Aotsukaa

a Division of Cardiology, Chiba Children's Hospital, Chiba City, Japan, b Division of Neurology, Chiba Children's Hospital, c Division of Metabolism, Chiba Children's Hospital

Correspondence to: Dr Yoshitomo Okajima, Division of Cardiology, Chiba Children's Hospital, 579-1 Heta-chou, Midori-ku, Chiba City 266-0007, Japan.

Accepted for publication 30 April 1998

Objective---To investigate cardiac function in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) and clarify the clinical features of cardiomyopathy in MELAS.
Patients---11 consecutive patients with MELAS (mean age at initial examination 11.3 years, range 4 to 16 years) were enrolled in the study. Six were followed for more than five years.
Results---On echocardiographic examination, three patients showed increased left ventricular end diastolic posterior wall thickness (LVPWTd), exceeding 140% of the normal value. Four patients, including these three, had an ejection fraction of less than 50%, and two also had increased left ventricular end diastolic volume (LVEDV) exceeding 140% of the normal value (%N). The LVPWTd%N was correlated positively with the LVEDV%N (R = 0.669, p < 0.05) and negatively with the ejection fraction (R = -0.6701, p < 0.05). One patient died of heart failure aged 22 years.
Conclusions---The cardiomyopathy in MELAS is characterised by an abnormally thick left ventricular wall with progressive dilatation and poor left ventricular contraction developing over several years, indicating hypertrophic cardiomyopathy advancing to dilated cardiomyopathy.

Keywords: MELAS;  cardiomyopathy

© 1998 by Heart
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