TY - JOUR
T1 - Early initiation of prostacyclin in portopulmonary hypertension
T2 - 10 Years of a transplant center's experience
AU - Awdish, Rana L A
AU - Cajigas, Hector R.
N1 - Funding Information:
Dr. Cajigas has served as a consultant for United Therapeutics Inc., Actelion and Gilead. He has served in the speaker’s bureau for Actelion and United Therapeutics Inc, received honoraria for service on Advisory boards from United Therapeutics, Actelion and Gilead. Dr. Cajigas has received Grants and research support from Pfizer, Actelion and United Therapeutics. Dr. Awdish has received Grants and research support from Pfizer, Actelion and United Therapeutics.
PY - 2013/12
Y1 - 2013/12
N2 - 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.
AB - 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.
KW - Model for end stage liver disease
KW - Orthotopic liver transplant
KW - Portopulmonary hypertension
KW - Prostacyclin
KW - Pulmonary arterial hypertension
KW - Transplant clearance
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U2 - 10.1007/s00408-013-9501-5
DO - 10.1007/s00408-013-9501-5
M3 - Article
C2 - 23979006
AN - SCOPUS:84890441273
SN - 0341-2040
VL - 191
SP - 593
EP - 600
JO - Pneumonologie. Pneumonology
JF - Pneumonologie. Pneumonology
IS - 6
ER -