Primary pulmonary hypertension with plexiform vascular changes in the lungs and liver cirrhosis is a rare combination of unclear pathogenesis. Until now, the real prevalence has not been known. The diagnosis of this association is usually made retrospectively. The criteria are morphological--that is, right ventricular hypertrophy and the characteristic pulmonary arterial lesions, as well as clinical--based on ECG and chest radiography. Between 1970 and 1977, two such cases have been found among a total of 11988 necropsies performed on adults. In the same necropsy series, 765 cases of liver cirrhosis were found. The prevalence of this combination is 0.26% of the cirrhosis and 0.016% of all necropsies of adults. This low prevalence raises serious doubts as to whether the association is more than coincidental.
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