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Circulation, Vol 70, 580-587, Copyright © 1984 by American Heart Association
V Fuster, PM Steele, WD Edwards, BJ Gersh, MD McGoon and RL Frye
A long-term retrospective follow-up study was made of 120 patients (33
male, 87 female patients) with primary pulmonary hypertension-- diagnosed
by strict clinical and hemodynamic criteria--to obtain a better
understanding of the natural history and possible pathogenetic mechanisms
of the disease. The mean age at diagnosis was 34 (3 to 64) years, but only
24 patients (21%) remained alive 5 years later. Lung tissue obtained at
autopsy from 56 patients revealed two major pathologic types:
thromboembolic pulmonary hypertension in 32 patients (57%) and plexogenic
pulmonary arteriopathy in 18 (32%). Thus, in more than half the patients
undergoing autopsy the major histologic feature was thrombi without any
evidence of plexiform lesions. The two groups were similar with respect to
their clinical and hemodynamic features and short survival. Of the
variables tested for prognostic importance by stepwise multivariate
analysis, only two were significant: pulmonary arterial oxygen saturation
(p less than .00001) and anticoagulant therapy (p = .01). Anticoagulant
therapy is recommended for patients with primary pulmonary hypertension.
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Primary pulmonary hypertension: natural history and the importance of thrombosis
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