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REVEAL Registry: One Year Outcome of Patients with Portopulmonary Hypertension (PoPH)

Michael Krowka


D. Miller

Robyn Barst


Michael McGoon


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Conference: 2010 International PHA Conference and Scientific Sessions

Release Date: 06.24.2010

Presentation Type: Abstracts

Krowka MJ1, Miller DP2, Barst RJ3, McGoon MD1.
1. Mayo Clinic, Rochester, MN, USA
2. ICON Clinical Research, Ellicott City, MD, USA
3. Columbia University College of Physicians & Surgeons, New York, NY, USA

BACKGROUNDThe Registry to EValuate Early And Long-term PAH Disease Management (REVEAL) is a multicenter, observational, US-based study evaluating demographics and management of patients diagnosed with pulmonary arterial hypertension (PAH). 3500 patients have been enrolled and will be followed for a minimum of five years from enrollment. The objectives of this study include the 1-year outcomes of the patients with a Group I diagnosis of PoPH.

METHODSThe 1-year outcomes (survival from time of enrollment and all cause hospitalization post-enrollment) of patients age ≥19 years with a diagnosis of PoPH were examined. These outcome results were compared to patients of age ≥19 years with idiopathic PAH (IPAH).

RESULTSOf the 2767 patients aged ≥19 years entered in the REVEAL Registry from March 30, 2006 through September 11, 2007, 154 patients had a diagnosis at enrollment of PoPH, and 19.5% of these patients were not on prostanoids, endothelin receptor antagonists or phosphodiesterase-5 inhibitors. The patients with PoPH had the following demographic characteristics: mean age at enrollment, 53 ± 10 years; female gender, 49%; newly diagnosed, 16%; time from diagnosis to enrollment, 27 ± 30 months; and NYHA functional class IV, 4%. Demographics for IPAH (n=1287) that were significantly different from PoPH included female gender, 78% (P<0.001); and time from diagnosis to enrollment, 38 ± 40 months (P=0.001). The 1-year post-enrollment survival outcome was significantly worse for PoPH vs IPAH (85% vs 93%, respectively; P<0.001), though the % patients who were free from hospitalization (all-cause) was not (62% vs 72%, respectively; P=0.142). Patients with PoPH were less likely to be treated with endothelin receptor antagonists than patients with IPAH (8% vs 47%, respectively; P<0.001).

CONCLUSIONS: Patients with PoPH have a relatively balanced gender distribution vs a female preponderance for patients with IPAH. Patients with PoPH have significantly lower survival at 1 year after enrollment than patients with IPAH.