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Portopulmonary hypertension

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 Society of Critical Care Medicine (SCCM 2020), the European Association for the Study of the Liver (EASL 2018), the International Liver Transplantation Society (ILTS 2016), and the American Association for the Study of Liver Diseases (AASLD 2014).
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Guidelines

1.Screening and diagnosis

Indications for testing
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

2.Classification and risk stratification

Severity assessment: 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

3.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

4.Diagnostic procedures

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

5.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

6.Therapeutic procedures

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

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  • ECMO

  • RV assist device

7.Perioperative care

Perioperative monitoring: obtain pulmonary artery catheter monitoring before and during liver transplantation in all adult patients.
B
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  • Perioperative medical therapy

  • Postoperative evaluation

8.Surgical interventions

Liver transplantation: as per EASL 2018 guidelines, consider performing liver transplantation if mean pulmonary arterial pressure is < 35 mmHg and RV function is preserved. Do not perform liver transplantation in patients with a mean pulmonary arterial pressure of ≥ 45 mmHg, irrespective of applied therapy.
B
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9.Follow-up and surveillance

Serial clinical assessment: obtain clinical monitoring of cardiac function and for complications of liver disease.
B