Pulmonary Hypertension as a Result of Asymptomatic Portosystemic Shunt

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This report describes a patient with severe pulmonary hypertension accompanied by a congenital intrahepatic portosystemic shunt. Primary pulmonary hypertension 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.