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Early Initiation of Prostacyclin in Portopulmonary Hypertension: 10 Years of a Transplant Center’s Experience

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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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Abbreviations

ALT:

Alanine aminotransferase

AST:

Aspartate aminotransferase

6MWD:

Six minute walk distance

CO:

Cardiac output

ETRA:

Endothelin receptor antagonist

IPAH:

Idiopathic pulmonary arterial hypertension

mPAP:

Mean pulmonary arterial pressure

MELD:

Model for end stage liver disease

OLT:

Orthotopic liver transplant

PAH:

Pulmonary arterial hypertension

PASP:

Pulmonary artery systolic pressure

PCWP:

Pulmonary artery occlusion pressure

PDEI:

Phosphodiesterase inhibitor

PoPH:

Portopulmonary hypertension

PVR:

Pulmonary vascular resistance

NYHA:

New York Heart Association

REVEAL:

Registry to Evaluate Early and Long-term PAH Disease Management

TPG:

Transpulmonary gradient

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Conflict of interest

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.

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Authors and Affiliations

  1. Pulmonary and Critical Care Medicine Division, Henry Ford Hospital, 2799 West Grand Boulevard, K-17, Detroit, MI, 48202, USA

    Rana L. A. Awdish & Hector R. Cajigas

Authors
  1. Rana L. A. Awdish
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  2. Hector R. Cajigas
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Correspondence to Rana L. A. Awdish.

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Awdish, R.L.A., Cajigas, H.R. Early Initiation of Prostacyclin in Portopulmonary Hypertension: 10 Years of a Transplant Center’s Experience. Lung 191, 593–600 (2013). https://doi.org/10.1007/s00408-013-9501-5

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  • DOI: https://doi.org/10.1007/s00408-013-9501-5

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