Summary
The histopathology of the lungs is described in 13 patients with pulmonary veno-occlusive disease. The patients were mostly children or young adults and there was no sex preference as is seen in primary pulmonary hypertension.
Characteristically the small pulmonary veins and venules were narrowed or occluded by intimal fibrosis, but larger veins were also affected. These obstructive lesions almost certainly resulted from organization of thrombi. Changes in the pulmonary arteries were generally secondary to obstruction of pulmonary venous flow but there was also a high incidence of thrombotic changes in the arteries.
Pulmonary parenchymal lesions commonly found included interstitial pneumonia and fibrosis, siderosis and cellular proliferation, together with increased activity of bronchial mucous glands and bronchial epithelial mucous cells. These changes may have indicated an infection, possibly of viral nature, as an aetiological factor in some of the patients. Consistent with such a cause is the common observation of an infectious febrile respiratory illness preceding the onset of symptoms due to pulmonary veno-occlusive disease.
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We are grateful to Dr. I. Anderson and Dr. J. B. MacGillivray, London, U.K., Dr. D. B. Brewer and Prof. R. C. Curran, Birmingham, U.K., Dr. J. E. Edwards, Saint Paul, Minn., U.S.A., Dr. E. L. Frensdorf, Amsterdam, Netherlands, Prof. D. Heath, Liverpool, U.K., Prof. R. E. B. Hudson, London, U.K., Dr. A. Rosenthal, Boston, Mass., U.S.A., Dr. P. G. I. Stovin, Papworth Everard, U.K. and Dr. R. Wollmann, Chicago, Ill., U.S.A., for submitting cases of pulmonary veno-occlusive disease to us. Technical aid in this study was given by Miss H. J. Dijk and Mr. R. Verhoeven.
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Wagenvoort, C.A., Wagenvoort, N. The pathology of pulmonary veno-occlusive disease. Virchows Arch. A Path. Anat. and Histol. 364, 69–79 (1974). https://doi.org/10.1007/BF01230858
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DOI: https://doi.org/10.1007/BF01230858