A patient with end-stage liver disease as a result of alpha1-antitripsin deficiency presented for orthotopic liver transplantation. The liver cirrhosis was complicated by portal hypertension and hepatopulmonary syndrome resulting in varicosities and severe hypoxia (room air oxygen saturation 69%). After transplantation, the hepatopulmonary syndrome improved but, over the next 14 months, the patient developed severe pulmonary hypertension. Six years posttransplantation, his room air oxygen saturation was 95% with pulmonary artery pressures of 109 mm Hg systolic and 26 mm Hg diastolic (mean 55 mm Hg) and a pulmonary vascular resistance 688 dynes x sec x cm.