Hepatopulmonary syndrome as the first and only manifestation of cirrhosis in a patient with hypopituitarism

BMJ Case Rep. 2021 Sep 20;14(9):e244805. doi: 10.1136/bcr-2021-244805.

Abstract

Hepatopulmonary syndrome (HPS) is characterised by the development of intrapulmonary arteriovenous blood shunts and vascular dilatation with consequent hypoxaemia, usually in the context of end-stage liver disease (ESLD). The estimated incidence of HPS in ESLD has been reported to be 13%-47%. Chronic liver disease has been described in patients with hypothalamic-pituitary dysfunction, mainly in the form of non-alcoholic fatty liver disease due to metabolic syndrome, with occasional progression to cirrhosis. We report a challenging case of a 27-year-old man with a background of hypopituitarism with no known liver disease who presented with progressive dyspnoea and hypoxaemia and was eventually diagnosed with severe HPS.

Keywords: cirrhosis; pituitary disorders; pulmonary hypertension; transplantation.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • End Stage Liver Disease*
  • Fibrosis
  • Hepatopulmonary Syndrome* / diagnosis
  • Hepatopulmonary Syndrome* / diagnostic imaging
  • Humans
  • Hypopituitarism* / complications
  • Hypopituitarism* / diagnosis
  • Liver Cirrhosis / complications
  • Male