Current status of hepatopulmonary syndrome in liver transplantation in Japan: a Japanese multicenter analysis

J Hepatobiliary Pancreat Sci. 2019 Jul;26(7):292-299. doi: 10.1002/jhbp.632. Epub 2019 Jun 10.

Abstract

Background: Hepatopulmonary syndrome (HPS) negatively affects the outcomes of deceased donor liver transplantation (LT).

Methods: We retrospectively reviewed the clinical records of patients with HPS who underwent LT and studied the impact of risk factors on clinical outcomes to determine strategies to overcome complications. Patients with symptoms of hypo-oxygenemia and a shunt ratio >15% on 99mTc-MAA lung perfusion scintigraphy were defined as having HPS.

Results: Forty-eight patients in 10 centers were enrolled. Diseases included biliary atresia, liver cirrhosis, non-alcoholic steatohepatitis, congenital hepatic fibrosis, and others. The length of ICU stay was 2-170 days. The respirator was used for 41.6% of patients on post-operative day (POD) 3 and 20.8% on POD 14. The patient survival rate was 87% at 1 year and 82% at 5 years. The causes of hospital mortality were sepsis, thrombotic microangiopathy, intracranial bleeding, pulmonary fibrosis, and transplant rejection. An amount of shunt ratio prior to LT was a significant risk factor for hospital mortality. Hypoxia from POD 3 to POD 14 was a risk factor for biliary stenosis. The shunt ratio of all surviving patients significantly improved.

Conclusion: Although LT is feasible for patients with HPS, early transplantation and avoiding hypo-oxygenemia immediately after transplantation are important.

Keywords: Hepatopulmonary syndrome; Liver transplantation.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Female
  • Hepatopulmonary Syndrome / complications*
  • Hepatopulmonary Syndrome / mortality
  • Hospital Mortality
  • Humans
  • Hypoxia / complications
  • Hypoxia / mortality
  • Infant
  • Japan
  • Liver Diseases / mortality
  • Liver Diseases / surgery*
  • Liver Transplantation* / mortality
  • Male
  • Middle Aged
  • Retrospective Studies
  • Survival Rate