Pulmonary hypertension complicating portal hypertension.
We report 9 patients with pulmonary hypertension complicating portal hypertension. The cause of portal hypertension was cirrhosis in 7 patients, nodular regenerative hyperplasia of the liver in 1, and portal vein obstruction in 1. Six patients had been treated by portal-systemic shunting before the clinical onset of pulmonary hypertension. The interval between the first manifestation of portal hypertension and the recognition of pulmonary hypertension ranged from 2 to 15 years. Histologic examination in 1 of these patients revealed medial hypertrophy, concentric intimal proliferation, and plexiform lesions affecting the small pulmonary arteries. Pulmonary hypertension might result from the effect of a vasoconstrictive agent on the small pulmonary arteries or of a substance toxic to the walls of these vessels that is produced in the splanchnic territory, destroyed by the liver in normal subjects, and reaches the pulmonary arteries through portal-systemic shunts in these patients.
D000328:Adult; D000368:Aged; D006332:Cardiomegaly / Q000175:diagnosis / Q000209:etiology; D004562:Electrocardiography; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications* / Q000628:therapy; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D008103:Liver Cirrhosis / Q000150:complications; D008168:Lung / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011167:Portacaval Shunt, Surgical / Q000009:adverse effects; D011169:Portal Vein; D011651:Pulmonary Artery / Q000000981:diagnostic imaging; D011859:Radiography; D013927:Thrombosis / Q000150:complications