Primary Pulmonary Hypertension: A Histopathologic Study of 80 Cases
Section snippets
METHODS
From the tissue registry at our institution, we identified 80 cases of primary pulmonary hypertension that had been diagnosed between 1930 and 1983. These 80 cases form the basis of our report and include 73 autopsies, 6 open-lung biopsy specimens, and 1 case with both biopsy and autopsy tissue; the clinical features of 53 of these cases are being reported in a separate communication.6 Excluded from our study were cases with congenital cardiac shunts, obstructive lesions of the left side of the
Histopathologic Classification.
The cases were grouped on the basis of the dominant structural abnormalities present. Thromboembolic pulmonary hypertension was characterized by thrombi of various ages and by eccentric intimal proliferation and fibrosis.2, 3, 4 Plexiform lesions were the histopathologic hallmark of primary plexogenic pulmonary arteriopathy, but concentric intimal proliferation and fibroelastosis were also highly suggestive of this disorder.2, 3, 4 When a case had both plexiform and thrombotic lesions, it was
DISCUSSION
The histopathologic types of clinical primary pulmonary hypertension were described in detail by the Wagenvoorts in 1970 in a classic study of 156 autopsy cases2 and in 1980 in an appraisal of 40 open-lung biopsy specimens.5 To these two elegant studies, we add our own observations based on a semiquantitative histopathologic study of 80 cases (Table 2).
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Mayo Graduate School of Medicine, Rochester, Minnesota. Present address: University of Iceland, Reykjavik, Iceland.