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[Hepatopulmonary syndrome].

G P Glattki1, C Maurer, N Satake

  • 1Abteilung für Pneumologie/Allergologie, Ruhrlandklinik Essen-Heidhausen.

Medizinische Klinik (Munich, Germany : 1983)
|November 2, 1999
PubMed
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Hepatopulmonary syndrome, a liver disease complication, involves lung vascular changes. Liver transplantation improved hypoxemia and pulmonary vascular dilatations in a patient with this condition.

Area of Science:

  • Hepatology
  • Pulmonology
  • Cardiology

Background:

  • A 60-year-old woman presented with progressive dyspnea and liver cirrhosis secondary to Hepatitis C.
  • Clinical manifestations included signs of chronic liver disease and respiratory failure, such as spider naevi, palmar erythema, clubbing, platypnea, and severe hypoxemia.

Observation:

  • The patient exhibited orthodeoxia, with arterial pO2 deteriorating in an upright position.
  • Transthoracic echocardiography suggested pulmonary vascular dilatations without septal defects.
  • Perfusion scans indicated abnormal pulmonary vascular passage, while imaging ruled out interstitial lung disease or anatomical shunts.

Findings:

  • The diagnosis was hepatopulmonary syndrome Type 1.
  • Following liver transplantation, the patient showed improved general condition and resolution of cutaneous signs.

Related Experiment Videos

  • Post-transplant, perfusion scans normalized, and orthodeoxia and hypoxemia significantly improved, indicating diminished pulmonary vascular dilatations.

    • Implications:

    • Liver transplantation is an effective treatment for hepatopulmonary syndrome.
    • Resolution of pulmonary vascular dilatations post-transplantation suggests reversibility of the condition.
    • This case highlights the importance of considering hepatopulmonary syndrome in patients with liver cirrhosis and unexplained hypoxemia.