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Comparative Study
. 2014 Dec;146(6):1494-1504.
doi: 10.1378/chest.13-3014.

Unique predictors of mortality in patients with pulmonary arterial hypertension associated with systemic sclerosis in the REVEAL registry

Affiliations
Comparative Study

Unique predictors of mortality in patients with pulmonary arterial hypertension associated with systemic sclerosis in the REVEAL registry

Lorinda Chung et al. Chest. 2014 Dec.

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.

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Figures

Figure 1 –
Figure 1 –
STROBE diagram of the Registry to Evaluate Early and Long-Term PAH Management (REVEAL) Registry patients used in this analysis. We included only patients with CTD-APAH who met the strict criteria of World Health Organization group 1 pulmonary arterial hypertension. CTD-APAH = pulmonary arterial hypertension associated with connective tissue disease; HRCT = high-resolution CT scan of the chest; ILD = interstitial lung disease; non-SSc-CTD = connective tissue disease other than systemic sclerosis; PCWP = pulmonary capillary wedge pressure; SSc = systemic sclerosis; TLC = total lung capacity.
Figure 2 –
Figure 2 –
Three-year survival curves in patients with SSc and non-SSc-CTD-APAH. A, Three-year survival from enrollment in the newly diagnosed SSc group was 51.2% ± 4.0% compared with 76.4% ± 4.6% in the non-SSc-CTD group (P < .001). B, Three-year survival from enrollment in the previously diagnosed SSc group was 61.4% ± 2.7% compared with 80.9% ± 2.7% in the non-SSc-CTD group (P < .001). See Figure 1 legend for expansion of abbreviations.
Figure 3 –
Figure 3 –
Predictors of mortality for patients with SSc-APAH and non-SSc-CTD-APAH using univariate Cox regression analyses. Unique predictors of mortality in the SSc group, but not the non-SSc group, included male sex, SBP ≤ 110 mm Hg, pericardial effusion, Dlco ≤ 32% predicted, mRAP > 20 mm Hg within 1 y, PVR > 32 WU, and newly diagnosed status. BNP levels < 50 pg/mL were protective in patients with SSc, but not in the non-SSc group. Higher GFR was protective in both groups. Mild to moderate ILD was the only feature that increased mortality in the non-SSc group but not in patients with SSc. 6MWD = 6-min walk distance; BNP = brain natriuretic peptide; DLCO = diffusion capacity of the lung for carbon monoxide; FC = functional class; GFR = glomerular filtration rate; HR = hazard ratio; mRAP = mean right atrial pressure; NYHA = New York Heart Association; PVR = pulmonary vascular resistance; SBP = systolic BP; WHO = World Health Organization; WU = Wood units. See Figure 1 legend for expansion of other abbreviations.

References

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