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.

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.

[1]  R. Virmani,et al.  Heterogeneity of pathologic lesions in familial primary pulmonary hypertension. , 1988, The American review of respiratory disease.

[2]  J. Rouleau,et al.  Familial pulmonary capillary hemangiomatosis resulting in primary pulmonary hypertension. , 1988, Annals of internal medicine.

[3]  E H Bergofsky,et al.  Primary pulmonary hypertension. A national prospective study. , 1987, Annals of internal medicine.

[4]  B. Brundage,et al.  Primary pulmonary hypertension: radiographic and scintigraphic patterns of histologic subtypes. , 1986, Annals of internal medicine.

[5]  D. Heath,et al.  Pathologic Study of Unexplained Pulmonary Hypertension , 1985 .

[6]  T. Takahashi,et al.  Pulmonary veno-occlusive disease: involvement of pulmonary arteries and review of the literature. , 1985, Human pathology.

[7]  W. Edwards,et al.  Primary pulmonary hypertension: a histopathologic study of 80 cases. , 1985, Mayo Clinic proceedings.

[8]  V. Fuster,et al.  Primary pulmonary hypertension: natural history and the importance of thrombosis. , 1984, Circulation.

[9]  J. Newman,et al.  Familial primary pulmonary hypertension: clinical patterns. , 1984, The American review of respiratory disease.

[10]  W. Mooi,et al.  Decreased numbers of pulmonary blood vessels: Reality or artifact? , 1983, The Journal of pathology.

[11]  G. Vawter,et al.  Early pulmonary vascular changes in congenital heart disease studied in biopsy tissue. , 1980, Human pathology.

[12]  C. Wagenvoort.  Lung biopsy specimens in the evaluation of pulmonary vascular disease. , 1980, Chest.

[13]  L. Reid,et al.  A morphometric study of regional variation in lung structure in infants with pulmonary hypertension and congenital cardiac defect. A justification of lung biopsy. , 1978, British heart journal.

[14]  W. Edwards,et al.  Clinical primary pulmonary hypertension: three pathologic types. , 1977, Circulation.

[15]  C. Wagenvoort,et al.  Primary Pulmonary Hypertension: A Pathologic Study of the Lung Vessels in 156 Clinically Diagnosed Cases , 1970 .