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Portopulmonary hypertension: a report from the US-based REVEAL Registry.
- Source :
-
CHEST . Apr2012, Vol. 141 Issue 4, p906-915. 10p. - Publication Year :
- 2012
-
Abstract
- <bold>Background: </bold>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).<bold>Methods: </bold>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).<bold>Results: </bold>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.<bold>Conclusions: </bold>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.<bold>Trial Registry: </bold>ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 00123692
- Volume :
- 141
- Issue :
- 4
- Database :
- Academic Search Index
- Journal :
- CHEST
- Publication Type :
- Academic Journal
- Accession number :
- 108174947
- Full Text :
- https://doi.org/10.1378/chest.11-0160