Primary pulmonary hypertension developing 11 years after a splenorenal shunt for portal hypertension in hepatic cirrhosis.

Objective : To describe a patient with pulmonary and portal hypertension and to review the pathogenesis and management of this condition. Patient : A 22-year-old woman with portal hypertension and liver cirrhosis who later developed pulmonary hypertension. Interventions : The patient received antibiotic therapy and underwent a splenectomy and proximal splenorenal shunt for portal hypertension. She later received hydralazine, digoxin and warfarin for pulmonary hypertension. Main outcome measures : After undergoing a splenorenal shunt the patient made a good recovery. However, she later developed pulmonary hypertension and'died after undergoing a hysterectomy and a bilateral salpingo-oophorectomy. Conclusions : The concomitant development of portal and pulmonary hypertension in the same patient has a poor prognosis. The treatment available for patients with these conditions is unsatisfactory and most patients eventually die as a result of cardiovascular disease. Cardiopulmonary and liver transplantation should be considered in suitable patients.