title: Early initiation of prostacyclin in portopulmonary hypertension: 10 years of a transplant center's experience.
abstract: BACKGROUND
  Portopulmonary hypertension (PoPH) is a subgroup of Group 1 pulmonary arterial hypertension (PAH) with particularly poor prognosis. Delay in initiation of parenteral therapy may be the reason for poor outcome.
  
  
  METHODS
  We conducted a prospective observational study of all patients with Group 1 PoPH evaluated at Henry Ford Hospital between January 2002 and July 2012. The cohort of the REVEAL Registry patients with PoPH was used as the comparator group. The patient survival rates at 5 years after diagnosis and 2 years after enrollment, treatment trends, and the freedom from all-cause hospitalization rates at 2 years after enrollment were compared using χ (2) analysis.
  
  
  RESULTS
  Twenty-one patients were enrolled in the PH Clinic with PoPH from January 2002 through July 2012. Our patients were significantly more likely to be on prostacyclin IV at 90 days as compared to REVEAL PoPH patients (67 vs. 31 %; p = 0.002). Despite this, early outcomes were not significantly different between the groups: 2-year survival from enrollment (70 and 67 %, respectively; p = 0.77) and 2-year freedom from hospitalization (35 vs. 49 %, respectively; p = 0.29). However, 5-year survival from time of diagnosis was significantly higher in our cohort of PoPH patients (71 and 40 %, respectively; p = 0.02).
  
  
  CONCLUSIONS
  Early initiation of parenteral prostacyclin therapy in PoPH patients at a single institution was associated with improved 5-year patient survival from diagnosis as compared to the REVEAL Registry of PoPH patients and allowed for clearance for transplant in 52 % of patients within 1 year.
authors: Pulmonary and Critical Care Medicine Division, Henry Ford Hospital, 2799 West Grand Boulevard, K-17, Detroit, MI, 48202, USA, Rawdish1@hfhs.org.
authors: Rana L A
authors: RL
authors: Awdish
authors: Hector R
authors: HR
authors: Cajigas
mesh_terms: D061605:Administration, Intravenous; D000328:Adult; D000368:Aged; D000959:Antihypertensive Agents / Q000008:administration & dosage*; D001794:Blood Pressure / Q000187:drug effects*; D016009:Chi-Square Distribution; D018572:Disease-Free Survival; D004334:Drug Administration Schedule; D061345:Early Medical Intervention; D011464:Epoprostenol / Q000008:administration & dosage*; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality / Q000503:physiopathology; D053208:Kaplan-Meier Estimate; D016031:Liver Transplantation; D008297:Male; D008824:Michigan; D008875:Middle Aged; D010359:Patient Readmission; D011446:Prospective Studies; D012042:Registries; D012307:Risk Factors; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage*
chemical_list: D000959:Antihypertensive Agents; D014665:Vasodilator Agents; D011464:Epoprostenol
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