Circulation, Vol 56, 884-888, Copyright © 1977 by American Heart Association
WD Edwards and JE Edwards
Clinically, there is a group of patients with elevated pulmonary arterial
pressure in whom the underlying cause is not apparent. The pulmonary
arterial wedge pressure is not elevated. For such cases, the designation of
primary pulmonary hypertension may be made clinically. From the clinical
categorization of primary pulmonary hypertension, three distinct pathologic
entities emerge, namely 1) plexogenic pulmonary arteriopathy, 2) recurrent
pulmonary thromboembolism, and 3) pulmonary veno-occlusive disease. The
plexogenic type is characterized initially by pulmonary arterial
vasoconstriction with medial hypertrophy. Secondary proliferative intimal
lesions, including the plexiform lesion, develop. Recurrent pulmonary
thromboembolism is characterized by the presence of arterial thrombi of
varying ages involving the microscopic-sized pulmonary arteries. Thrombi
may be embolic in nature or may develop in situ. Pulmonary veno-occlusive
disease is characterized by obstructive lesions of pulmonary veins and
venules. The clinical presentation of the three pathologic types may be so
similar that definitive diagnosis depends upon histologic examination of
the lung from tissue obtained either by biopsy or at necropsy.
ARTICLES
Clinical primary pulmonary hypertension: three pathologic types
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