Refractory hypoxemia caused by hepatopulmonary syndrome: A case report

Morgen L. Govindan*, Kevin W. Kuo, Maryam Ghadimi Mahani, Thomas P. Shanley

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

2 Scopus citations


Introduction: Hepatopulmonary syndrome is a clinical syndrome that can affect patients of all ages with liver disease and is more common in children with biliary atresia. Contrast echocardiography is the test of choice to diagnose the presence of intrapulmonary vascular dilatation. The established treatment for hepatopulmonary syndrome is liver transplantation. Case presentation: We present the case of an 8-month-old Caucasian baby boy with a history of biliary atresia, polysplenia, and interrupted inferior vena cava who presented with hypoxemia and cyanosis that progressed rapidly. A chest computed tomography angiogram revealed significant dilatation of the pulmonary vasculature, prompting further evaluation and diagnosis of hepatopulmonary syndrome with contrast echocardiography. He was maintained on a milrinone infusion while awaiting liver transplantation. His hypoxemia improved slowly following liver transplantation, requiring tracheostomy and prolonged ventilator dependence. Conclusions: Hepatopulmonary syndrome should be included in the differential for progressive hypoxemia in children with liver disease, particularly those with biliary atresia. Imaging with chest computed tomography angiogram and contrast echocardiography should be considered in cases of unexplained refractory hypoxemia.

Original languageEnglish (US)
Article number418
JournalJournal of Medical Case Reports
Issue number1
StatePublished - Dec 10 2014


  • Contrast echocardiography
  • Hepatopulmonary syndrome
  • Pediatric
  • Refractory hypoxemia

ASJC Scopus subject areas

  • Medicine(all)


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