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Hepatopulmonary syndrome

Key sources
The following summarized guidelines for the evaluation and management of hepatopulmonary syndrome are prepared by our editorial team based on guidelines from the Society of Critical Care Medicine (SCCM 2020), the European Association for the Study of the Liver (EASL 2018), and the International Liver Transplantation Society (ILTS 2016).


1.Screening and diagnosis

Indications for testing: as per EASL 2018 guidelines, evaluate patients with the hallmarks of chronic liver disease for HPS in the presence of tachypnea and polypnea, digital clubbing, and/or cyanosis.
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  • Diagnostic criteria

2.Classification and risk stratification

Severity assessment: consider using the ERS severity classification of HPS in research and clinical settings.

3.Diagnostic investigations

Pulse oximetry: as per EASL 2018 guidelines, obtain pulse oximetry to screen for HPS in adult patients.

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  • Blood gas analysis

  • Contrast echocardiography

  • Macroaggregated albumin lung perfusion scan

  • Pulmonary angiography

4.Respiratory support

Oxygen therapy: as per SCCM 2020 guidelines, provide supportive care with supplemental oxygen in the management of patients with HPS, pending possible liver transplantation.

5.Medical management

Pharmacotherapy: as per EASL 2018 guidelines, insufficient evidence regarding the use of medical therapy for the treatment of patients with HPS.

6.Therapeutic procedures

Transjugular intrahepatic portosystemic shunt: as per EASL 2018 guidelines, insufficient evidence to recommend TIPS placement for the treatment of patients with HPS.

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  • Coil embolization

  • ECMO

7.Perioperative care

Intraoperative oxygen monitoring
Obtain continuous monitoring of mixed venous oxygen saturation. Consider performing venovenous bypass if the mixed venous oxygen saturation falls < 65% on vascular exclusion of the liver.
Recognize that intraoperative oxygenation is not adversely affected by different anesthetic deliveries.

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  • Postoperative oxygen therapy

  • Postoperative fluid management

  • Postoperative imaging

8.Surgical interventions

Liver transplantation
As per EASL 2018 guidelines:
Evaluate patients with HPS and PaO2 < 60 mmHg for liver transplantation since it is the only effective treatment for HPS.
Obtain arterial blood gas analysis every 6 months to facilitate prioritization of liver transplantation since severe hypoxemia (PaO2 < 45-50 mmHg) is associated with increased post-liver transplantation mortality.

9.Specific circumstances

Pediatric patients: consider correcting congenital portosystemic shunts in pediatric patients.