Natural Course of Cardiomyopathy in Duchenne Muscular Dystrophy : SYMPOSIUM ON SECONDARY MYOCARDIAL DISEASE : 44th Scientific Session of the Japanise Circulation Society

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In order to examine the natural course of cardiomyopathy in Duchenne muscular dystrophy, we performed (1) long-term observation of electrocardiograms (ECGs) in 40 patients and (2) assessment of left ventricular function in three age groups of 33 patients. The observation periods of ECGs ranged between 1 and 9 years, with average of 5.4 years. QRS abnormalities in leads V_1, I, V_5 and aV_F were used to detect the cardiac lesions in the posterobasal, high lateral, lateral and inferior portions of the left ventricle. The cardiac involvement was observed to occur in most of the patients during the observation period. In 75% of the patients, the cardiac involvement progressed in its location and/or severity. In many cases, the cardiac lesion commenced in the posterobasal portion and extended to the lateral wall of the left ventricle. Left ventricular function was examined using echocardiography and mechanocardiography in 3 different age groups of the patients. The stroke index, ejection fraction, mean rate of circumferential shortening (mVcF), posterior wall excursion, mean posterior wall velocity in systole and in diastole and the PEP/ET ratio were compared to those of the age-matched normals. In patients aged 6-10 years, the stroke index was significantly decreased. In patients aged 11-15 years, all the echocardiographic measurements except mVcF were decreased and the PEP/ET ratio was increased. In patients aged 16 years or older, all the echocardiographic measurements were decreased and the PEP/ET ratio was increased. It is suggested that most of the patients develop progressive cardiac lesions during the Life. The cardiac lesions may commence at the posterobasal portion and further involve the lateral wall of the left ventricle. The left ventricular function may also be involved progressively in these patients.
社団法人日本循環器学会の論文
1979-12-20

Natural Course of Cardiomyopathy in Duchenne Muscular Dystrophy : SYMPOSIUM ON SECONDARY MYOCARDIAL DISEASE : 44th Scientific Session of the Japanise Circulation Society

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