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Original Research |

Portopulmonary HypertensionPortopulmonary Hypertension in the REVEAL Registry: A Report From the US-Based REVEAL Registry

Michael J. Krowka, MD, FCCP; Dave P. Miller, MS; Robyn J. Barst, MD, FCCP; Darren Taichman, MD, PhD, FCCP; Raed A. Dweik, MD, FCCP; David B. Badesch, MD, FCCP; Michael D. McGoon, MD, FCCP
Author and Funding Information

From the Mayo Clinic (Drs Krowka and McGoon), Rochester, MN; ICON Late Phase and Outcomes Research (Mr Miller), San Francisco, CA; the Columbia University College of Physicians and Surgeons (Dr Barst), New York, NY; the University of Pennsylvania Medical Center (Dr Taichman), Philadelphia, PA; the Cleveland Clinic (Dr Dweik), Cleveland, OH; and the University of Colorado (Dr Badesch), Denver, CO.

Correspondence to: Michael J. Krowka, MD, FCCP, Pulmonary and Critical Care Medicine, Mayo Clinic Transplant Center, Mayo Clinic, 200 1st St South West, Rochester, MN 55905; e-mail: krowka.michael@mayo.edu


For editorial comment see page 840

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).


© 2012 American College of Chest Physicians


Chest. 2012;141(4):906-915. doi:10.1378/chest.11-0160
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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.

Trial registry:  ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov

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