Chest
Original ResearchPulmonary Vascular DiseasePortopulmonary Hypertension: A Report From the US-Based REVEAL Registry
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.
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