PULMONARY-HYPERTENSION COMPLICATING PORTAL-HYPERTENSION

Publisher Website
Google Scholar
Abstract
The cases of 9 patients with pulmonary hypertension complicating portal hypertension are reported. The cause of portal hypertension was cirrhosis in 7 patients, nodular regenerative hyperplasia of the liver in one, and portal vein obstruction in one. Six patients were treated by portal-systemic shunting before the clinical onset of pulmonary hypertension. The interval between the 1st manifestation of portal hypertension and the recognition of pulmonary hypertension ranged 2-15 yr. Histologic examination in 1 patient 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.