Chest
Volume 146, Issue 6, December 2014, Pages 1494-1504
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Original Research: Pulmonary Vascular Disease
Unique Predictors of Mortality in Patients With Pulmonary Arterial Hypertension Associated With Systemic Sclerosis in the REVEAL Registry

https://doi.org/10.1378/chest.13-3014Get rights and content
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Abstract

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

ABBREVIATION

6MWD
6-min walk distance
BNP
brain natriuretic peptide
CTD
connective tissue disease
CTD-APAH
pulmonary arterial hypertension associated with connective tissue disease
Dlco
diffusion capacity of the lung for carbon monoxide
FC
functional class
HR
hazard ratio
ILD
interstitial lung disease
IPAH
idiopathic pulmonary arterial hypertension
mRAP
mean right atrial pressure
non-SSc-CTD
connective tissue disease other than systemic sclerosis
NT-pro-BNP
N-terminal-pro-brain natriuretic peptide
NYHA
New York Heart Association
PAH
pulmonary arterial hypertension
PVR
pulmonary vascular resistance
REVEAL Registry
Registry to Evaluate Early and Long-Term PAH Management
RHC
right-sided heart catheterization
SBP
systolic BP
SSc
systemic sclerosis
SSc-APAH
pulmonary arterial hypertension associated with systemic sclerosis
WHO
World Health Organization
WU
Wood units

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This is an open access article distributed under the terms of the Creative Commons Attribution-Noncommercial License (http://creativecommons.org/licenses/by-nc/3.0/), which permits unrestricted use, distribution, and reproduction to noncommercial entities, provided the original work is properly cited. Information for reuse by commercial entities is available online.

FUNDING/SUPPORT:Actelion Pharmaceuticals US Inc is the sponsor of REVEAL Registry and provided funding and support for the analysis presented.