SEVERE PULMONARY-HYPERTENSION AND AMELIORATION OF HEPATOPULMONARY SYNDROME AFTER LIVER-TRANSPLANTATION

A patient with end-stage liver disease as a result of alpha(1)-antitri psin deficiency presented for orthotopic liver transplantation. The li ver cirrhosis was complicated by portal hypertension and hepatopulmona ry syndrome resulting in varicosities and severe hypoxia (room air oxy gen saturation 69%). After transplantation, the hepatopulmonary syndro me improved but, over the next 14 months, the patient developed severe pulmonary hypertension. Six years posttransplantation, his room air o xygen saturation was 95% with pulmonary artery pressures of 109 mm Hg systolic and 26 mm Hg diastolic (mean 55 mm Hg) and a pulmonary vascul ar resistance 688 dynes.sec.cm.(5) Copyright (C) 1998 by the American Association for the Study of Liver Diseases.