Severe portopulmonary hypertension after liver transplantation in a patient with preexisting hepatopulmonary syndrome

Background: Portopulmonary hypertension and hepatopulmonary syndrome have b een considered mutually exclusive pulmonary vascular disorders in liver dis ease states. Methods: This current report describes a middle-aged patient, a candidate f or liver transplantation, diagnosed with hepatopulmonary syndrome on the ba sis of clinical, echocardiographic and gas exchange criteria. Unusually hig h pulmonary pressures were observed at liver transplantation, performed 6 m onths after the initial diagnosis of hepatopulmonary syndrome, Three months later, the patient developed severe pulmonary hypertension and died of rig ht ventricular failure during a second attempted liver transplantation. Pos t-mortem histologic findings in the lung confirmed the presence of plexogen ic pulmonary arteriopathy. Conclusion: This case illustrates the potential occurrence of hepatopulmona ry syndrome and portopulmonary hypertension in the same patient, suggesting that the presence of hepatopulmonary syndrome may not preclude the develop ment of portopulmonary hypertension.