Hepatopulmonary syndrome: favorable outcomes in the MELD exception era

Hepatology. 2013 Jun;57(6):2427-35. doi: 10.1002/hep.26070. Epub 2013 Feb 15.


Hepatopulmonary syndrome (HPS) is a pulmonary vascular disorder occurring as a consequence of advanced liver disease, characterized by hypoxemia due to intrapulmonary vascular dilatations. HPS independently increases mortality, regardless of the cause or severity of liver disease. Liver transplantation (LT) improves survival in HPS. We present the largest consecutive series of HPS patients specifically addressing long-term survival relative to the degree of hypoxemia and the era in which LT was conducted. We evaluated 106 HPS patients at the Mayo Clinic from 1986 through 2010. Survival was assessed using Kaplan-Meier methodology. LT was accomplished in 49 HPS patients. Post-LT survival (1, 3, 5, and 10 years) did not differ between groups based on baseline partial pressure of arterial oxygen (PaO2 ) obtained at the time of HPS diagnosis. Improvements in overall survival at 1, 3, and 5 years post-LT in those HPS patients transplanted after January 1 2002 (n = 28) (92%, 88%, and 88%, respectively) as compared with those transplanted prior to that time (n = 21) (71%, 67%, and 67%, respectively) did not reach statistical significance (5-year P = 0.09). Model for Endstage Liver Disease (MELD) exception to facilitate LT was granted to 21 patients since January 1 2002 with post-LT survival of 19/21 patients and one wait-list death.

Conclusion: Long-term outcome after LT in HPS is favorable, with a trend towards improved survival in the MELD exception era since 2002 as compared to earlier HPS transplants. Survival after LT was not associated with PaO2 levels at the time of HPS diagnosis. (HEPATOLOGY 2012).

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Cerebrovascular Circulation
  • Child
  • Female
  • Follow-Up Studies
  • Hepatopulmonary Syndrome / complications
  • Hepatopulmonary Syndrome / diagnostic imaging
  • Hepatopulmonary Syndrome / mortality
  • Hepatopulmonary Syndrome / surgery*
  • Humans
  • Hypoxia / etiology
  • Liver Transplantation*
  • Lung / diagnostic imaging
  • Male
  • Middle Aged
  • Minnesota / epidemiology
  • Pulmonary Circulation
  • Radionuclide Imaging
  • Severity of Illness Index
  • Technetium Tc 99m Aggregated Albumin
  • Young Adult


  • Technetium Tc 99m Aggregated Albumin