title: Primary pulmonary hypertension: an unusual case associated with extrahepatic portal hypertension.
abstract: A patient with both extrahepatic portal hypertension and primary pulmonary hypertension is reported. The pulmonary hypertension developed without a surgical portal-systemic shunt, and at autopsy there was no evidence of a large spontaneous shunt. This association of pulmonary arterial hypertension and portal venous hypertension without either intrinsic liver disease or a large portal-systemic anastomosis has not been reported previously. Our case supports the concept that portal hypertension with its attendant portal-systemic collateral circulation may be the major predisposing cause of pulmonary hypertension both in intrinsic liver disease and extrahepatic portal vein obstruction.
mesh_terms: D000293:Adolescent; D006801:Humans; D006975:Hypertension, Portal / Q000473:pathology*; D006976:Hypertension, Pulmonary / Q000473:pathology*; D008099:Liver / Q000473:pathology; D008297:Male; D008642:Mesenteric Veins / Q000473:pathology; D011169:Portal Vein / Q000473:pathology; D011651:Pulmonary Artery / Q000473:pathology; D013162:Splenic Vein / Q000473:pathology; D013927:Thrombosis / Q000473:pathology