Circulation, Vol 57, 1122-1129, Copyright © 1978 by American Heart Association
SK Sanyal, WW Johnson, MK Thapar and SE Pitner
Electrocardiographic abnormalities were identified in 63 (84%) of 75
patients with Duchenne's progressive muscular dystrophy. A tall R wave over
V1 with an abnormal R/S ratio was seen in 64% of the patients, a deep and
narrow Q wave greater than 4 mm over leads I, V5, and V6 in 44%, sinus
tachycardia in 32% and right axis deviation in 16%. Other ECG abnormalities
included an abnormal PV1 index in 14% of patients and a short P-R interval
in 6%. Ultrastructural characteristics of the heart were determined for two
patients with characteristic electrocardiographic abnormalities. Common to
both hearts was a total loss of thick as well as thin myofilaments, which
gave a "moth-eaten" appearance to the myofiber. This feature, combined with
preservation of the transverse tubular system, formed the most
characteristic ultrastructural finding and was seen most consistently in
the posterobasal area of the left ventricle. Alterations of Z-band
material; accumulation of mitochondria, occasionally containing
electron-dense bodies and showing loss or discontinuity of cristae;
dilatation of sarcoplasmic reticulum with striking ectasia of cisternae;
depletion of glycogen particles; a paucity of lipoid or lipochrome
granules; and the absence of virus-like particles were other consistent
ultrastructural features. Comparison of skeletal and cardiac muscle
disclosed identical subcellular changes. These observations support the
contention that the distinctive ECG pattern associated with Duchenne's
dystrophy results from multifocal degenerative changes involving
myocardium, predominantly the posterobasal region of the left ventricle and
the posterior papillary muscle.
ARTICLES
An ultrastructural basis for electrocardiographic alterations associated with Duchenne's progressive muscular dystrophy
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