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

Chest, 2012-04, Vol.141 (4), p.906-915 [Peer Reviewed Journal]

2015 INIST-CNRS ;ISSN: 0012-3692 ;EISSN: 1931-3543 ;DOI: 10.1378/chest.11-0160 ;PMID: 21778257 ;CODEN: CHETBF

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Title:
Portopulmonary Hypertension: A Report From the US-Based REVEAL Registry
Author: KROWKA, Michael J ; MILLER, Dave P ; BARST, Robyn J ; TAICHMAN, Darren ; DWEIK, Raed A ; BADESCH, David B ; MCGOON, Michael D
Subjects: Biological and medical sciences ; Cardiology. Vascular system ; Female ; Gastroenterology. Liver. Pancreas. Abdomen ; Hemodynamics ; Hospitalization ; Humans ; Hypertension, Portal - complications ; Hypertension, Portal - mortality ; Hypertension, Portal - physiopathology ; Hypertension, Portal - therapy ; Hypertension, Pulmonary - complications ; Hypertension, Pulmonary - mortality ; Hypertension, Pulmonary - physiopathology ; Hypertension, Pulmonary - therapy ; Liver Diseases - complications ; Liver Function Tests ; Liver. Biliary tract. Portal circulation. Exocrine pancreas ; Male ; Medical sciences ; Middle Aged ; Other diseases. Semiology ; Pneumology ; Registries ; United States
Is Part Of: Chest, 2012-04, Vol.141 (4), p.906-915
Description: 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). 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). 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. 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. ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov.
Publisher: Northbrook, IL: American College of Chest Physicians
Language: English
Identifier: ISSN: 0012-3692
EISSN: 1931-3543
DOI: 10.1378/chest.11-0160
PMID: 21778257
CODEN: CHETBF
Source: MEDLINE
Alma/SFX Local Collection

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

2015 INIST-CNRS ;ISSN: 0012-3692 ;EISSN: 1931-3543 ;DOI: 10.1378/chest.11-0160 ;PMID: 21778257 ;CODEN: CHETBF

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