Document Detail

Histopathology of primary pulmonary hypertension. A qualitative and quantitative study of pulmonary blood vessels from 58 patients in the National Heart, Lung, and Blood Institute, Primary Pulmonary Hypertension Registry.
MedLine Citation:
PMID:  2805258     Owner:  NLM     Status:  MEDLINE    
Qualitative and quantitative studies were performed on pulmonary blood vessels in lung tissue obtained by biopsy, pneumonectomy, or autopsy from 58 patients in the Registry of Primary Pulmonary Hypertension sponsored by the Heart, Lung, and Blood Institute of the National Institutes of Health. In 49 patients (84%), the hypertensive vascular disease involved predominantly or exclusively muscular pulmonary arteries and arterioles. In each of these 49 patients, pulmonary artery medial hypertrophy was observed, and in 48 patients, it was also associated with intimal or luminal lesions. On the basis of the predominant histopathologic features, 25 of the 48 patients were classified as having pulmonary arteriopathy with plexiform lesions characterized by a combination of concentric laminar intimal fibrosis, eccentric intimal fibrosis, and plexiform lesions; in nine of these 25, recanalized thrombi were also present. Pulmonary arteriopathy with thrombotic lesions, defined by the presence of both eccentric intimal fibrosis and recanalized thrombi but without plexiform lesions, was observed in 19 patients. Intimal fibrosis, either concentric or eccentric, without plexiform or thrombotic lesions was found in four patients. Among the remaining nine patients in the Registry, pulmonary veno-occlusive disease was present in seven and chronic pulmonary venous hypertension in one. Pulmonary blood vessels were microscopically normal in a lung biopsy specimen from another patient. In general, patients with plexiform lesions and those with veno-occlusive disease had a much poorer prognosis than patients with thrombotic lesions. The present study shows the existence of several distinct histopathologic patterns of pulmonary vascular disease in individuals with primary pulmonary hypertension diagnosed by standardized clinical and laboratory criteria.
G G Pietra; W D Edwards; J M Kay; S Rich; J Kernis; B Schloo; S M Ayres; E H Bergofsky; B H Brundage; K M Detre
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Publication Detail:
Type:  Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Circulation     Volume:  80     ISSN:  0009-7322     ISO Abbreviation:  Circulation     Publication Date:  1989 Nov 
Date Detail:
Created Date:  1989-12-18     Completed Date:  1989-12-18     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0147763     Medline TA:  Circulation     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1198-206     Citation Subset:  AIM; IM    
Division of Lung Diseases, National Heart, Lung, and Blood Institute, Bethesda, Maryland.
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MeSH Terms
Hypertension, Pulmonary / pathology*
National Institutes of Health (U.S.)
Pulmonary Artery / pathology*
Pulmonary Embolism / pathology
Pulmonary Veins / pathology*
Pulmonary Veno-Occlusive Disease / pathology
United States
Grant Support
Comment In:
Circulation. 1989 Nov;80(5):1486-8   [PMID:  2805280 ]

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