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For further information, see CMDT Part 16-11: Cirrhosis

Key Features

  • Characteristic triad

    • Chronic liver disease

    • Increased alveolar-arterial gradient on room air

    • Intrapulmonary vascular dilatations or arteriovenous communications that result in a right-to-left intrapulmonary shunt

Clinical Findings

  • Dyspnea (platypnea) and arterial deoxygenation (orthodeoxia) are greater in the upright position than in the recumbent position

  • Suspect hepatopulmonary syndrome in a cirrhotic patient with a pulse oximetry level ≤ 96%

  • Elevated levels of von Willebrand factor antigen in affected patients suggests that endothelial dysfunction may have role

Diagnosis

  • Contrast-enhanced echocardiography is a sensitive screening test for detecting pulmonary vascular dilatations, whereas macroaggregated albumin lung perfusion scanning is more specific and may be used to confirm the diagnosis

  • High-resolution CT may be useful for detecting dilated pulmonary vessels that may be amenable to embolization

Treatment

  • Medical therapy has been disappointing

  • However, intravenous methylene blue, oral garlic powder, oral norfloxacin, and mycophenolate mofetil may improve oxygenation by inhibiting nitric oxide–induced vasodilatation and angiogenesis

  • Pentoxifylline may prevent hepatopulmonary syndrome by inhibiting production of tumor necrosis factor

  • Long-term oxygen therapy is recommended for severely hypoxemic patients

  • Hepatopulmonary syndrome may reverse with liver transplantation

  • Postoperative morbidity and mortality from severe hypoxemic respiratory failure are increased when the preoperative arterial oxygen tension is < 44 mm Hg (5.9 kPa) or with substantial intrapulmonary shunting

  • In cases confirmed by right-sided heart catheterization, treatment with the following may reduce pulmonary hypertension and thereby facilitate liver transplantation

    • Epoprostenol, iloprost, or treprostinil (the latter two are easier to administer)

    • Bosentan (no longer used because of potential hepatotoxicity), ambrisentan, or macitentan

    • Sildenafil, tadalafil or vardenafil

    • Selexipag

    • Riociguat

  • Beta-blockers worsen exercise capacity and are contraindicated

  • Calcium channel blockers should be used with caution because they may worsen portal hypertension

  • Liver transplantation is contraindicated in patients with moderate to severe pulmonary hypertension (mean pulmonary pressure > 35 mm Hg)

  • Transjugular intrahepatic portosystemic shunt (TIPS) may provide palliation in patients with hepatopulmonary syndrome awaiting transplantation

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