Plexogenic pulmonary arteriopathy and liver cirrhosis.
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.
D000368:Aged; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D008103:Liver Cirrhosis / Q000150:complications*; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery / Q000473:pathology; D011660:Pulmonary Heart Disease / Q000150:complications; D014652:Vascular Diseases / Q000150:complications / Q000473:pathology