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Portopulmonary hypertension

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of portopulmonary hypertension are prepared by our editorial team based on guidelines from the European Association for the Study of the Liver (EASL 2024,2018), the Society of Critical Care Medicine (SCCM 2020), the International Liver Transplantation Society (ILTS 2016), and the American Association for the Study of Liver Diseases (AASLD 2014).
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Screening and diagnosis

Indications for testing
As per ILTS 2016 guidelines:
Obtain Doppler transthoracic echocardiography to screen for POPH in patients with portal hypertension being candidates for TIPS insertion or liver transplantation.
B
Obtain repeated screening for POPH in patients with portal hypertension while waiting for liver transplantation.
B
Recognize that female gender and autoimmune liver diseases are risk factors for POPH, but do not use these for screening.
B
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  • Diagnostic criteria

  • Differential diagnosis

Classification and risk stratification

Severity assessment: as per ILTS 2016 guidelines, consider describing the severity of POPH in terms of mean pulmonary artery pressure (assuming increased pulmonary vascular resistance) as follows:
Situation
Guidance
Mild
25-34
Moderate
35-44
Severe
≥ 45
B

Diagnostic investigations

Echocardiography
As per EASL 2018 guidelines:
Obtain Doppler TTE to screen for POPH in patients deemed potential recipients for TIPS or liver transplantation.
B
Obtain repeated echocardiography in patients with POPH listed for transplantation.
B

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  • Vasoreactivity testing

Diagnostic procedures

Right heart catheterization: as per EASL 2018 guidelines, perform right heart catheterization in patients with a positive screening echocardiogram.
B

Medical management

Pulmonary arterial hypertension-specific pharmacotherapy: as per SCCM 2020 guidelines, consider treating POPH with agents approved for pulmonary arterial hypertension in patients with mean pulmonary artery pressure > 35 mmHg.
C

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  • Medications to avoid

Therapeutic procedures

TIPS: as per EASL 2018 guidelines, do not perform TIPS placement in patients with POPH.
D

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  • ECMO

  • RV assist device

Perioperative care

Perioperative monitoring: as per ILTS 2016 guidelines, obtain pulmonary artery catheter monitoring before and during liver transplantation in all adult patients.
B
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  • Perioperative medical therapy

  • Postoperative evaluation

Surgical interventions

Liver transplantation: as per EASL 2024 guidelines, recognize that POPH alone is not an indication for liver transplantation in patients with compensated liver disease.
B
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Follow-up and surveillance

Clinical follow-up: as per ILTS 2016 guidelines, obtain clinical monitoring of cardiac function and for complications of liver disease.
B