Early initiation of prostacyclin in portopulmonary hypertension

10 Years of a transplant center's experience

Rana L A Awdish*, Hector R. Cajigas

*Corresponding author for this work

Research output: Contribution to journalArticle

16 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)593-600
Number of pages8
JournalLung
Volume191
Issue number6
DOIs
StatePublished - Dec 1 2013

Fingerprint

Epoprostenol
Hypertension
Transplants
Survival
Registries
Hospitalization
Pulmonary Hypertension
Observational Studies
Therapeutics
Survival Rate
Prospective Studies

Keywords

  • Model for end stage liver disease
  • Orthotopic liver transplant
  • Portopulmonary hypertension
  • Prostacyclin
  • Pulmonary arterial hypertension
  • Transplant clearance

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Awdish, Rana L A ; Cajigas, Hector R. / Early initiation of prostacyclin in portopulmonary hypertension : 10 Years of a transplant center's experience. In: Lung. 2013 ; Vol. 191, No. 6. pp. 593-600.
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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.",
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Early initiation of prostacyclin in portopulmonary hypertension : 10 Years of a transplant center's experience. / Awdish, Rana L A; Cajigas, Hector R.

In: Lung, Vol. 191, No. 6, 01.12.2013, p. 593-600.

Research output: Contribution to journalArticle

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.

PY - 2013/12/1

Y1 - 2013/12/1

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

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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

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JO - Lung

JF - Lung

SN - 0341-2040

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