TY - JOUR
T1 - Unique Predictors of Mortality in Patients With Pulmonary Arterial Hypertension Associated With Systemic Sclerosis in the REVEAL Registry
AU - Chung, Lorinda
AU - Farber, Harrison W.
AU - Benza, Raymond
AU - Miller, Dave P.
AU - Parsons, Lori
AU - Hassoun, Paul M.
AU - McGoon, Michael
AU - Nicolls, Mark R.
AU - Zamanian, Roham T.
N1 - Funding Information:
Financial/nonfinancial disclosures: The authors have reported toCHESTthe following conflicts of interest: Dr Chung has received research support funding from Gilead Sciences, Inc; United Therapeutics Corp; Pfizer, Inc; and Actelion Pharmaceuticals Ltd, and has served on the Advisory Board for Gilead Sciences, Inc. Dr Farber has served as a consultant for Gilead Sciences, Inc, Actelion Pharmaceuticals Ltd, Bayer, United Therapeutics Corp, and Bristol-Myers Squibb; has served on the speakers bureau for Actelion Pharmaceuticals Ltd, Gilead Sciences, Inc, and Bayer; and has received grant support from Gilead Sciences, Inc and United Therapeutics Corp. Dr Benza has grant support from Actelion Pharmaceuticals Ltd and is a member of the Steering Committee for the REVEAL Registry. Mr Miller is an employee of ICON Clinical Research, a company that receives funding from Actelion Pharmaceuticals Ltd and acts as a BioStatistical CRO for the REVEAL Registry, as well as received funding from other pharmaceutical companies. Ms Parsons is an employee of ICON Clinical Research, a company that receives funding from Actelion Pharmaceuticals Ltd and acts as a BioStatistical CRO for the REVEAL Registry, as well as received funding from other pharmaceutical companies. Dr Hassoun has received research funding support from Actelion/CoTherix and is on the Advisory Board for Novartis. Dr McGoon has received research funding from Gilead Sciences, Inc and Medtronic, Inc and has served on steering committees for Gilead Sciences, Inc and Lung Rx, LLC and has participated on clinical end-point committees in studies sponsored by Actelion Pharmaceuticals Ltd. He is on a Data Safety Monitoring Board for a study sponsored by Gilead Sciences, Inc and has received honoraria for his service on the REVEAL Registry Steering Committee, which is supported by Actelion Pharmaceuticals Ltd. Dr Zamanian has received research funding support through the Enteligence-Actelion career development research grant and has served as a consultant to United Therapeutics Corporation and Gilead Sciences, Inc. Dr Nicolls has reported that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.
Publisher Copyright:
© 2014 The American College of Chest Physicians
PY - 2014/12/1
Y1 - 2014/12/1
N2 - BACKGROUND:Patients with pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc-APAH) experience higher mortality rates than patients with idiopathic disease and those with other connective tissue diseases (CTD-APAH). We sought to identify unique predictors of mortality associated with SSc-APAH in the CTD-APAH population. METHODS:The Registry to Evaluate Early and Long-Term PAH Management (REVEAL Registry) is a multicenter, prospective US-based registry of patients with previously and newly diagnosed (enrollment within 90 days of diagnostic right-sided heart catheterization) PAH. Cox regression models evaluated all previously identified candidate predictors of mortality in the overall REVEAL Registry population to identify significant predictors of mortality in the SSc-APAH (n = 500) vs non-SSc-CTD-APAH (n = 304) populations. RESULTS:Three-year survival rates in the previously diagnosed and newly diagnosed SSc-APAH group were 61.4% ± 2.7% and 51.2% ± 4.0%, respectively, compared with 80.9% ± 2.7% and 76.4% ± 4.6%, respectively, in the non-SSc-CTD-APAH group (P <.001). In multivariate analyses, men aged > 60 years, systolic BP (SBP) ≤ 110 mm Hg, 6-min walk distance (6MWD) < 165 m, mean right atrial pressure (mRAP) > 20 mm Hg within 1 year, and pulmonary vascular resistance (PVR) > 32 Wood units remained unique predictors of mortality in the SSc-APAH group; 6MWD ≥ 440 m was protective in the non-SSc-CTD-APAH group, but not the SSc-APAH group. CONCLUSIONS:Patients with SSc-APAH have higher mortality rates than patients with non-SSc-CTD-APAH. Identifying patients with SSc-APAH who are at a particularly high risk of death, including elderly men and patients with low baseline SBP or 6MWD, or markedly elevated mRAP or PVR, will enable physicians to identify patients who may benefit from closer monitoring and more aggressive treatment. TRIAL REGISTRY:ClinicalTrials.gov; No.: NCT00370214; URL:www.clinicaltrials.gov
AB - BACKGROUND:Patients with pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc-APAH) experience higher mortality rates than patients with idiopathic disease and those with other connective tissue diseases (CTD-APAH). We sought to identify unique predictors of mortality associated with SSc-APAH in the CTD-APAH population. METHODS:The Registry to Evaluate Early and Long-Term PAH Management (REVEAL Registry) is a multicenter, prospective US-based registry of patients with previously and newly diagnosed (enrollment within 90 days of diagnostic right-sided heart catheterization) PAH. Cox regression models evaluated all previously identified candidate predictors of mortality in the overall REVEAL Registry population to identify significant predictors of mortality in the SSc-APAH (n = 500) vs non-SSc-CTD-APAH (n = 304) populations. RESULTS:Three-year survival rates in the previously diagnosed and newly diagnosed SSc-APAH group were 61.4% ± 2.7% and 51.2% ± 4.0%, respectively, compared with 80.9% ± 2.7% and 76.4% ± 4.6%, respectively, in the non-SSc-CTD-APAH group (P <.001). In multivariate analyses, men aged > 60 years, systolic BP (SBP) ≤ 110 mm Hg, 6-min walk distance (6MWD) < 165 m, mean right atrial pressure (mRAP) > 20 mm Hg within 1 year, and pulmonary vascular resistance (PVR) > 32 Wood units remained unique predictors of mortality in the SSc-APAH group; 6MWD ≥ 440 m was protective in the non-SSc-CTD-APAH group, but not the SSc-APAH group. CONCLUSIONS:Patients with SSc-APAH have higher mortality rates than patients with non-SSc-CTD-APAH. Identifying patients with SSc-APAH who are at a particularly high risk of death, including elderly men and patients with low baseline SBP or 6MWD, or markedly elevated mRAP or PVR, will enable physicians to identify patients who may benefit from closer monitoring and more aggressive treatment. TRIAL REGISTRY:ClinicalTrials.gov; No.: NCT00370214; URL:www.clinicaltrials.gov
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U2 - 10.1378/chest.13-3014
DO - 10.1378/chest.13-3014
M3 - Article
C2 - 24992469
AN - SCOPUS:84987678141
VL - 146
SP - 1494
EP - 1504
JO - Diseases of the chest
JF - Diseases of the chest
SN - 0012-3692
IS - 6
ER -