Hepatopulmonary syndrome is an increasingly recognized clinical entity (in 10% to 15% of patients with cirrhosis), characterized by abnormalities of arterial oxygenation in pa-tients with chronic liver disease and/or portal hypertension. The pathophysiology of this syndrome involves intrapulmonary vascular dilatation in the absence of architectural damage. These vascular abnormalities consist of precapilary dilatation, direct arteriovenous communications, and dilated pleural ves-sels. Intrapulmonary vascular dilatation is detected by contrast echocardiography, which shows delayed visualization of mi-crobubbles in the left heart chambers. The vascular dilatation leads to impaired oxygen transfer from alveoli to the central stream of red blood cells within capillaries, with a resulting "functional" intrapulmonary right-to-left shunt that improves with 100% oxygen. Clinical features range from subclinical abnormalities in gas exchange to profound hypoxemia causing dyspnea at rest. Patients often require supplemental oxygen and have significant limitations in their performance of usual daily activities. No proven medical therapy exists. As in hepa-torenal syndrome, hepatopulmonary syndrome is a functional disorder that reverses in most patients after liver transplanta-tion.

Hepatic Transplantation Liver transplantation is a high-ly successful procedure in patients with progressive, advanced, and otherwise untreatable liver disease. Advances surgical techniques and supportive care, the use of cyclosporine and tacrolimus for immunosuppression, and careful selection of pa-tients have all contributed to the encouraging results of liver transplantation. From 70 % to 80 % of patients undergoing liv-er transplantation survive at least 3 years, usually with good quality of life. The most common indication for liver trans-plantation in the United States is chronic liver disease resulting from hepatitis C virus infection. Other liver diseases for which transplantation is commonly performed include cirrhosis from alcoholic liver disease, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis. Patients with hepatitis B undergo liver transplantation if they do not have hepatitis e antigen and/or hepatitis B virus DNA in their seri-urn. Hepatitis B immunoglobulin is given after liver transplan-tation to help prevent recurrence. Trials evaluating the efficacy of nucleoside analogues in lowering replication of hepatitis B virus and in facilitating liver transplantation are under way. Excellent results have also been obtained in selected patients with fulminant hepatic failure. Liver transplantation for malig-nant hepatobiliary disease has been less successful because of recurrent disease in the transplanted liver.

The timing of liver transplantation presents a particular challenge. Liver-assist devices for temporary support are cur-rently being evaluated. The survival of ambulatory patients undergoing liver transplantation electively is greater than that of those who are critically ill at the time of the operation. Thus, liver transplantation is usually considered when the pa-tient has refractory ascites and/or spontaneous bacterial peri-tonitis, recurrent variceal hemorrhage, intractable pruritus, or an unacceptable quality of life.

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