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Therapy Insight: hepatopulmonary syndrome and orthotopic liver transplantation

Abstract

Hepatopulmonary syndrome (HPS)—a pulmonary complication observed in patients who have chronic liver disease and/or portal hypertension—is attributed to intrapulmonary vascular dilatation and induces severe hypoxemia. HPS is mainly detected when patients are included on the waiting list for orthotopic liver transplantation (OLT) and can be diagnosed by blood gas analysis, transthoracic contrast-enhanced echocardiography or body scan with 99mTc-labeled macroaggregated albumin perfusion. When the partial pressure of arterial oxygen (PaO2) is ≥80 mmHg, it is unlikely that the patient has HPS. When the PaO2 is <80 mmHg, imaging techniques should be used to confirm or exclude pulmonary vascular dilatation. When a diagnosis of HPS is confirmed, knowing the degree of hypoxemia is crucial for optimum patient management. Patients who have a PaO2 ≥50 mmHg but <60 mmHg should be prioritized for OLT. This procedure is not indicated for patients with a PaO2 between 60 mmHg and 80 mmHg, although follow-up every 3 months is recommended to detect any deterioration of the PaO2. A PaO2 of <50 mmHg might preclude OLT, because mortality and morbidity after OLT are greatly increased in these patients.

Key Points

  • Hepatopulmonary syndrome is defined by hypoxemia, intrapulmonary vascular dilatation, and liver disease

  • Careful evaluation of these three criteria is important

  • When the diagnosis of hepatopulmonary syndrome is certain, the severity of the syndrome should be regularly assessed

  • The priority for liver allocation in patients with hepatopulmonary syndrome and a partial pressure of arterial oxygen (PaO2) in the range 50–60 mmHg should be confirmed

  • The decision to perform liver transplantation in patients with hepatopulmonary syndrome and a PaO2 below 50 mmHg is made on a case-by-case basis

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Figure 1: An illustration of a positive test result for hepatopulmonary syndrome by contrast-enhanced echocardiography.
Figure 2: The algorithm used for the screening of patients on the waiting list for orthotopic liver transplantation and the therapeutic decisions made thereafter.

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Correspondence to Catherine M Pastor.

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The authors declare no competing financial interests.

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Pastor, C., Schiffer, E. Therapy Insight: hepatopulmonary syndrome and orthotopic liver transplantation. Nat Rev Gastroenterol Hepatol 4, 614–621 (2007). https://doi.org/10.1038/ncpgasthep0965

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  • DOI: https://doi.org/10.1038/ncpgasthep0965

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