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Book

Hepatopulmonary Syndrome

Kamna Bansal  1 , Meghana Gore  2 , Lafaine M. Grant  3 , Sahil Mittal  4
In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan.
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Book

Hepatopulmonary Syndrome

Kamna Bansal et al.
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Excerpt

Hepatopulmonary syndrome was first proposed in 1977 based on autopsy and clinical findings. Autopsies showed dilated pulmonary vasculature in patients with liver cirrhosis and were thought to cause some of the pulmonary manifestations seen in patients with chronic liver disease. Hepatopulmonary syndrome is defined as reduced arterial oxygen saturation due to dilated pulmonary vasculature in the presence of advanced liver disease or portal hypertension.

Diagnostic Criteria

The diagnostic criteria for hepatopulmonary syndrome include:

  1. Partial pressure of oxygen (PaO2): Defined as <80 mm Hg while breathing room air or alveolar-arterial oxygen gradient (A-aO2), ≥15 mm Hg while breathing room air. In patients older than 64, A-aO2 >20 mm Hg is considered diagnostic (these patients should be resting in a seated position).

  2. Pulmonary vascular dilatation: Dilatation as shown by a positive contrast-enhanced echocardiography or by radioactive lung-perfusion scanning (demonstrating a brain shunt fraction of >6%).

  3. Portal hypertension: Elevated pressure within the portal venous system with or without cirrhosis.

The severity of hepatopulmonary syndrome is classified based on the following PaO2 levels:

  1. Mild: PaO2 ≥80 mm Hg with A-aO2 ≥15 mm Hg while breathing room air

  2. Moderate: PaO2 ≥60 mm Hg to <80 mm Hg with A-aO2 ≥15 mm Hg while breathing room air

  3. Severe: PaO2 ≥50 mm Hg to <60 mm Hg with A-aO2 ≥15 mm Hg while breathing room air

  4. Very severe: PaO2 <50 mm Hg with A-aO2 ≥15 mm Hg while breathing room air, or PaO2 <300 mm Hg while breathing 100% oxygen

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Conflict of interest statement

Disclosure: Kamna Bansal declares no relevant financial relationships with ineligible companies.

Disclosure: Meghana Gore declares no relevant financial relationships with ineligible companies.

Disclosure: Lafaine Grant declares no relevant financial relationships with ineligible companies.

Disclosure: Sahil Mittal declares no relevant financial relationships with ineligible companies.

References

    1. Kennedy TC, Knudson RJ. Exercise-aggravated hypoxemia and orthodeoxia in cirrhosis. Chest. 1977 Sep;72(3):305-9. - PubMed
    1. Krowka MJ, Fallon MB, Kawut SM, Fuhrmann V, Heimbach JK, Ramsay MA, Sitbon O, Sokol RJ. International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension. Transplantation. 2016 Jul;100(7):1440-52. - PubMed
    1. Rodríguez-Roisin R, Krowka MJ. Hepatopulmonary syndrome--a liver-induced lung vascular disorder. N Engl J Med. 2008 May 29;358(22):2378-87. - PubMed
    1. Fallon MB, Krowka MJ, Brown RS, Trotter JF, Zacks S, Roberts KE, Shah VH, Kaplowitz N, Forman L, Wille K, Kawut SM, Pulmonary Vascular Complications of Liver Disease Study Group Impact of hepatopulmonary syndrome on quality of life and survival in liver transplant candidates. Gastroenterology. 2008 Oct;135(4):1168-75. - PMC - PubMed
    1. Schenk P, Fuhrmann V, Madl C, Funk G, Lehr S, Kandel O, Müller C. Hepatopulmonary syndrome: prevalence and predictive value of various cut offs for arterial oxygenation and their clinical consequences. Gut. 2002 Dec;51(6):853-9. - PMC - PubMed

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