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Background

Owing to the success of orthotopic liver transplantation (OLT), there has been increasing recognition of the importance of pulmonary vascular complications of hepatic disease states. Such vascular complications, namely hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN), are commonly present as dyspnoea and are not always easily distinguished from nonpulmonary symptoms caused by manifestations of advanced liver disease (such as anaemia, ascites and muscle wasting). More importantly, since the late 1980s, experience has taught that such complications influence survival and candidacy for OLT. Currently, OLT is the only effective treatment for improving outcome in patients with HPS, a life-threatening condition whose prevalence can approach 20% in some series of patients awaiting OLT. PPHTN, pulmonary arterial hypertension (PAH) occurring in the setting of liver …