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Hepatopulmonary syndrome and portopulmonary hypertension


  • Conflict of Interest: No conflict of interest has been declared by the author.

Dr Patrick S. Kamath, Mayo Clinic College of Medicine, Miles and Shirley Fiterman Center for Digestive Diseases, Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905, USA. Email:


The pulmonary complications of end-stage liver disease include hepatopulmonary syndrome and portopulmonary hypertension. The etio-pathogenesis of these conditions is as yet unclear. Hepatopulmonary syndrome is a gas exchange abnormality and usually manifests as hypoxemia secondary to intra-pulmonary vascular shunts. These shunts can be demonstrated by echocardiography using agitated saline injections, and quantitated by lung perfusion scans. Liver transplantation is the treatment of choice for hepatopulmonary syndrome, and there are no effective pharmacological therapies. Portopulmonary hypertension is a hemodynamic problem which manifests as fatigue and right sided cardiac failure. Several vasoactive agents have been used to lower mean pulmonary arterial pressures. Portopulmonary hypertension is a relative contraindication to liver transplantation.