Primary pulmonary hypertension: an unusual case associated with extrahepatic portal hypertension.

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.