Pulmonary hypertension as a result of asymptomatic portosystemic shunt

This report describes a patient with severe pulmonary hypertension accompan ied by a congenital intrahepatic portosystemic shunt. Primary pulmonary hyp ertension was suspected initially because none of the classic symptoms of a portosystemic shunt were present. Physicians should note that disorders of the portal system may cause pulmonary hypertension even in the absence of symptoms suggesting liver disease.