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Circulation, Vol 53, 483-490, Copyright © 1976 by American Heart Association
BH Bulkley, RL Ridolfi, WR Salyer and GM Hutchins
The nature, prevalence, functional significance, and indeed existence of
myocardial disease in progressive systemic sclerosis (PSS) has been
debated. In this study the clinical and pathological features of 52
autopsied patients were analyzed in an attempt to resolve these questions.
A distinctive focal myocardial lesion ranging from contraction band
necrosis to replacement fibrosis throughout both ventricular walls was
present in 23 patients who had widely patent extramural coronary arteries.
There were no morphologic abnormalities of the intramyocardial coronary
arteries to account for these lesions. Comparing those patients having
severe (13), mild (10), or no (24) PSS myocardial lesions, and patent
extramural coronary arteries, there were no major differences in age, sex,
frequency and severity of pulmonary, renal or hypertensive disease which
could account for the myocardial necrosis and fibrosis. The three groups
did differ, however, with regard to clinical cardiac abnormalities:
ventricular arrhythmias and conduction disturbances were six and two times
as frequent, respectively, in those with severe myocardial PSS compared to
the other two groups. A pattern of primary myocardial disease with
intractable congestive heart failure resulted from severe myocardial PSS in
four patients, angina pectoris with normal coronary arteries was associated
with the severe myocardial lesion in three patients, and sudden death in
five. The occurrence of contraction band necrosis suggests that the
myocardial damage in PSS might be due to intermittent vascular spasm of the
type recognized in the digits and possibly kidneys and lungs, i.e., an
intramyocardial Raynaud's phenomenon. The findings in our patients clearly
show that myocardial progressive systemic sclerosis is a distinct entity
with relatively frequent occurrence which may lead to arrhythmias,
congestive heart failure, angina pectoris with normal coronary arteries and
sudden death.
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Myocardial lesions of progressive systemic sclerosis. A cause of cardiac dysfunction
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