Abstract

Table 1 summarizes the results of five large pathology series published during the past 20 years. The great majority of patients with PPH had wide­ spread abnormalities only in the arterial side of the pulmonary circulation at autopsy or lung biopsy and were classified as having pulmonary hypertensive arteriopathy. In a small percentage of cases, the hypertensive changes did involve primarily the venous pulmonary circulation, and the patients were classi­ fied as having pulmonary veno-ocelusive disease. In some cases, the diagnosis of PPH could not be confirmed histopathologically.

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