Hepatopulmonary syndrome

Pascal Fauconnet, Vincent Ho, Catherine Pastor, Eduardo Schiffer

    Research output: Chapter in Book / Conference PaperChapter

    Abstract

    Hepatopulmonary syndrome (HPS) is a pulmonary complication observed in patients with chronic liver disease and/or portal hypertension. HPS is attributable to an intrapulmonary vascular dilatation that induces severe hypoxemia. Considering the favourable long-term survival of HPS patients as well as the reversal of the syndrome with a functional liver graft, HPS is now an indication for liver transplantation (LT). Both patients with mild cirrhosis who present with shortness of breath and all patients with end stage liver disease who are candidates for liver transplantation should undergo screening for HPS. Blood gas analysis and contrast-enhanced echocardiography are two main screening tools, together with lung function tests that can also detect additional pulmonary diseases that can contribute to impaired oxygenation. If the partial pressure of oxygen in arterial blood (PaO2) is > 80 mmHg, HPS can be excluded and no other investigation is needed. However, when PaO2 is ≤ 80 mmHg, contrast-enhanced echocardiography should be performed to obtain evidence of or to exclude pulmonary vascular dilatation. When the contrast-enhanced echocardiography is negative, HPS is excluded and no follow-up is needed. When the contrast-enhanced echocardiography is positive and PaO2 < 60 mmHg, patients should obtain a severity score that provides them with a reasonable probability of being transplanted within 3 months. In mild-to-moderate HPS (60 mmHg ≤ PaO2 < 80 mmHg), periodic follow-up is recommended every 3 months to detect deterioration of PaO2. Although no intra-operative death has been directly attributed to HPS, the immediate post-LT oxygenation worsens in relation to the volume overload, and infections are commonly observed after LT surgery. Mechanical ventilation is often prolonged and the stay in the Intensive Care Unit is extended. a high postoperative mortality (mostly within 6 months) is observed in this group of patients in comparison with non-HPS patients. However, the recovery of an adequate PaO2 within 12 months after LT explains the similar outcomes of HPS and non-HPS patients following LT.
    Original languageEnglish
    Title of host publicationCardiovascular Diseases and Liver Transplantation
    EditorsZoka Milan
    Place of PublicationU.K.
    PublisherNova
    Pages179-192
    Number of pages14
    ISBN (Print)9781611229103
    Publication statusPublished - 2011

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