title: Predictors of Waitlist Mortality in Portopulmonary Hypertension.
abstract: The current Organ Procurement Transplantation Network policy grants Model for End-Stage Liver Disease (MELD) exception points to patients with portopulmonary hypertension (POPH), but potentially important factors, such as severity of liver disease and pulmonary hypertension, are not included in the exception score, and may affect survival. The purpose of this study was to identify significant predictors of waitlist mortality in patients with POPH.
  
  
  
  We performed a retrospective cohort study of patients in the Organ Procurement and Transplantation Network database with hemodynamics consistent with POPH (defined as mean pulmonary arterial pressure >25 mm Hg and pulmonary vascular resistance [PVR] ≥240 dynes·s·cm) who were approved for a POPH MELD exception between 2006 and 2014. Using a Cox proportional hazards model, we identified predictors of waitlist mortality (or removal for clinical deterioration).
  
  
  
  One hundred ninety adults were included. Age (hazard ratio [HR], 1.04; 95% confidence interval [CI], 1.00-1.08; P = 0.0499), initial native MELD score (HR, 1.11; 95% CI, 1.05-1.17; P < 0.001), and initial PVR (HR, 1.12 per 100 dynes·s·cm; 95% CI, 1.02-1.23; P = 0.02) were the only significant univariate predictors of waitlist mortality and remained significant predictors in a multivariate model, which had a c-statistic of 0.71. PVR and mean pulmonary arterial pressure were not significant predictors of posttransplant mortality.
  
  
  
  Both the severity of liver disease and POPH (as assessed by MELD and PVR, respectively) were significantly associated with waitlist, but not posttransplant, mortality in patients with approved MELD exceptions for POPH. Both factors should potentially be included in the POPH MELD exception score to more accurately reflect waitlist mortality risk.
authors.affiliation: Department of Medicine, Massachusetts General Hospital Boston, MA USA.
authors.forename: Hilary M
authors.initials: HM
authors.lastname: DuBrock
authors.affiliation: Department of Medicine, Perelman School of Medicine at the University of Pennsylvania Philadelphia, PA USA.
authors.forename: David S
authors.initials: DS
authors.lastname: Goldberg
authors.affiliation: Department of Surgery, Baylor College of Medicine Houston TX USA.
authors.forename: Norman L
authors.initials: NL
authors.lastname: Sussman
authors.affiliation: Department of Medicine, University of Texas Southwestern Medical Center Dallas, TX USA.
authors.forename: Sonja D
authors.initials: SD
authors.lastname: Bartolome
authors.affiliation: Department of Surgery, Division of Transplantation, Penn State Milton S. Hershey Medical Center Hershey, PA USA.
authors.forename: Zakiyah
authors.initials: Z
authors.lastname: Kadry
authors.affiliation: Department of Medicine, Henry Ford Hospital Detroit, MI USA.
authors.forename: Reena J
authors.initials: RJ
authors.lastname: Salgia
authors.affiliation: Department of Surgery, Transplantation and Immunology, Yale University New Haven, CT USA.
authors.forename: David C
authors.initials: DC
authors.lastname: Mulligan
authors.affiliation: Department of Health Sciences Research, Mayo Clinic Rochester, MN USA.
authors.forename: Walter K
authors.initials: WK
authors.lastname: Kremers
authors.affiliation: Department of Medicine, Perelman School of Medicine at the University of Pennsylvania Philadelphia, PA USA.
authors.forename: Steven M
authors.initials: SM
authors.lastname: Kawut
authors.affiliation: Department of Medicine, Mayo Clinic Rochester, MN USA.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Krowka
authors.affiliation: Department of Medicine, Massachusetts General Hospital Boston, MA USA.
authors.forename: Richard N
authors.initials: RN
authors.lastname: Channick
mesh_terms: D062186:Arterial Pressure; D002423:Cause of Death; D016009:Chi-Square Distribution; D016208:Databases, Factual; D003661:Decision Support Techniques; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000401:mortality* / Q000503:physiopathology / Q000601:surgery; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000401:mortality* / Q000503:physiopathology / Q000601:surgery; D053208:Kaplan-Meier Estimate; D008107:Liver Diseases / Q000150:complications / Q000401:mortality* / Q000503:physiopathology / Q000601:surgery; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D017082:Portal Pressure; D011237:Predictive Value of Tests; D016016:Proportional Hazards Models; D011651:Pulmonary Artery / Q000503:physiopathology; D011652:Pulmonary Circulation; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D012720:Severity of Illness Index; D013997:Time Factors; D009927:Tissue and Organ Procurement; D016896:Treatment Outcome; D014655:Vascular Resistance; D014850:Waiting Lists / Q000401:mortality*