Abstract
The hepatopulmonary syndrome (HPS) occurs in as many as 15–20% of patients with cirrhosis; mortality is significantly increased compared to cirrhotic patients without HPS. The only proven effective therapy for HPS is orthotopic liver transplantation (OLT), which should be considered when severe hypoxemia is present. The natural history of HPS without liver transplantation is dismal. While post-OLT mortality is increased in patients with HPS relative to that reported in non-HPS patients, overall outcomes are favorable in properly selected patients. The higher mortality associated with HPS has led to the policy of increasing priority for OLT in selected HPS patients through a Model for End-Stage Liver Disease (MELD) score exception. There is currently no established protocol to screen for HPS in OLT candidates. However, a resting PaO2 < 65–60 mmHg identifies patients who qualify, or who may sufficiently deteriorate over a short time frame to qualify, for MELD exception criteria. In patients with HPS awaiting OLT, no specific therapies are available to improve intrapulmonary vasodilatation. The perioperative management of HPS patients presents particular clinical challenges.
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