Chest
Volume 141, Issue 4, April 2012, Pages 906-915
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Original Research
Pulmonary Vascular Disease
Portopulmonary Hypertension: A Report From the US-Based REVEAL Registry

https://doi.org/10.1378/chest.11-0160Get rights and content

Background

We evaluated survival and hospitalization rates in patients with group 1 portopulmonary hypertension (PoPH) in the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL Registry).

Methods

The REVEAL Registry is a multicenter, observational, US-based study evaluating demographics and management of patients with pulmonary arterial hypertension (PAH). Outcomes were examined using Kaplan-Meier time-to-event estimates and compared with patients with idiopathic PAH (IPAH) or familial PAH (FPAH).

Results

One hundred seventy-four patients with PoPH were enrolled in the REVEAL Registry (IPAH/FPAH; n = 1,478) from March 2006 to December 2009. Mean age was 53 ± 10 years, 52% were female, 32% were newly diagnosed, and 6% were New York Heart Association/World Health Organization functional class IV. Outcome parameters were worse for PoPH vs IPAH/FPAH, respectively: 2-year survival from enrollment (67% vs 85%, P < .001), 5-year survival from time of diagnosis (40% vs 64%, P < .001), and 2-year freedom from all-cause hospitalization (49% vs 59%, P = .019). However, despite worse outcomes, hemodynamic parameters at diagnosis were better for PoPH vs IPAH/FPAH, respectively: mean pulmonary artery pressure (49 mm Hg vs 53 mm Hg, P < .001), mean right atrial pressure (9 mm Hg vs 10 mm Hg, P = .005), pulmonary vascular resistance (8 Wood units vs 12 Wood units, P < .001), and cardiac output (5 L/min vs 4 L/min, P < .001). Compared with patients with IPAH/FPAH, patients with PoPH were less likely to be on a PAH-specific therapy at enrollment (P < .001), suggesting potential delays in therapy for patients with PoPH.

Conclusions

Patients with PoPH had significantly poorer survival and all-cause hospitalization rates compared with patients with IPAH/FPAH, despite having better hemodynamics at diagnosis. Further studies should investigate such outcomes and differences in treatment patterns.

Section snippets

Materials and Methods

One of the prespecified objectives of the REVEAL Registry was to evaluate differences in patient outcomes among the group 1 subgroups. The objective of the current analysis was to describe demographics, medical history, and outcomes of patients with PoPH enrolled in the REVEAL Registry using a cohort of REVEAL Registry patients with IPAH or familial PAH (FPAH) as a comparator population.

The design and inclusion criteria for the REVEAL Registry have been previously described.14 Patients in the

Patient Demographic, Hemodynamic, and Functional Characteristics

This analysis included 174 patients with PoPH and 1,478 patients with IPAH (n = 1,392) or FPAH (n = 85). The 174 patients with PoPH were enrolled at 36 REVEAL Registry sites. Twenty-six patients with PCWP > 15 mm Hg were excluded from these analyses. Separate analyses including these patients did not affect results or conclusions (data not shown). At enrollment, the PoPH and IPAH/FPAH subgroups were similar in age, proportion of newly diagnosed patients, 6-min walk test distance, and New York

Discussion

The REVEAL Registry includes the largest cohort of patients with PoPH followed prospectively. We observed key differences in the hemodynamic profiles, functional characteristics, treatment patterns, and outcomes between patients with PoPH and those with IPAH/FPAH.

Hemodynamic parameters at the time of diagnosis were better in the PoPH cohort vs the IPAH/FPAH cohort (ie, lower mPAP, PVR, and mRAP, and higher cardiac output). In addition, at the time of enrollment, mRAP and PVR remained

Conclusion

In a large cohort of patients with PoPH enrolled in the REVEAL Registry, survival from time of diagnosis, time of enrollment, and after the first hospitalization from enrollment was worse than that observed for patients with IPAH/FPAH. These differences were independent of the superior hemodynamic parameters of patients with PoPH at both the time of diagnostic RHC and at enrollment. Different treatment patterns, delayed initiation of PAH treatment in PoPH, and other hepatic-related

Acknowledgments

Author contributions: Dr Krowka: contributed to the study design; collection, analysis, and interpretation of data; drafting and critical review of the manuscript; and has seen and approved the final version.

Mr Miller: contributed to the study design; collection, analysis, and interpretation of data; drafting and critical review of the manuscript; and has seen and approved the final version.

Dr Barst: contributed to the study design; collection, analysis, and interpretation of data; drafting and

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Funding/Support: Funding for the REVEAL Registry is provided by Actelion Pharmaceuticals US, Inc.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).

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