Circulation, Vol 54, 348-356, Copyright © 1976 by American Heart Association
TN James
Pheochromocytoma may cause sudden and unexpected death. In this study of
three fatal cases of pheochromocytoma the small pulmonary arteries were
narrowed by a variety of chronic and acute processes which included medial
hypertrophy and fibrosis, endothelial proliferation and fibrosis, and
endothelial cellular edema; within the sinus node, atrioventricular (A-V)
node and His bundle of all three cases there was focal degeneration and
fibrosis similar to that also observed throughout the ventricular
myocardium; and in addition to the focal narrowing of many small coronary
arteries produced by medial hypertrophy, intimal fibrosis, and
fibromuscular dysplasia, there were also focal aggregations of platelets
clogging the lumen and occasionally mixed with a varying amount of fibrin.
In patients known to have pheochromocytoma it may be necessary to direct
new attention to the possibility of abnormal pulmonary vascular resistance,
instability of normal cardiac rhythm, and perhaps difficulty in restoring
it when disordered and to the effects of platelet aggregations, both
causing acute obstruction and possibly contributing to the pathogenesis of
chronic vascular disease.
ARTICLES
De subitaneis mortibus. XIX. On the cause of sudden death in pheochromocytoma, with special reference to the pulmonary arteries, the cardiac conduction system, and the aggregation of platelets
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