Comparison of pre- and postoperative clinical and hemodynamic parameters.

<p>Comparison of pre- and postoperative clinical and hemodynamic parameters.</p

Predictive factors of hemodynamic normalization^*.

<p>Predictive factors of hemodynamic normalization^{<a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0198198#t004fn002" target="_blank">*</a>}.</p

Flow chart of the study population.

<p>Flow chart of the study population.</p

Predictive factors of hemodynamic improvement^*.

<p>Predictive factors of hemodynamic improvement^{<a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0198198#t005fn002" target="_blank">*</a>}.</p

Predictive factors of functional improvement^*.

<p>Predictive factors of functional improvement^{<a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0198198#t006fn002" target="_blank">*</a>}.</p

Survival curves according to the optimal cut-off value derived by ROC analysis for preoperative pulmonary vascular resistance (PVR).

<p>Survival curves according to the optimal cut-off value derived by ROC analysis for preoperative pulmonary vascular resistance (PVR).</p

Predictive factors of mortality.

<p>Predictive factors of mortality.</p

Baseline patient characteristics.

<p>Baseline patient characteristics.</p

Clinical phenotypes and survival of pre-capillary pulmonary hypertension in systemic sclerosis

<div><p>Pre-capillary pulmonary hypertension (PH) in systemic sclerosis (SSc) is a heterogeneous condition with an overall bad prognosis. The objective of this study was to identify and characterize homogeneous phenotypes by a cluster analysis in SSc patients with PH. Patients were identified from two prospective cohorts from the US and France. Clinical, pulmonary function, high-resolution chest tomography, hemodynamic and survival data were extracted. We performed cluster analysis using the k-means method and compared survival between clusters using Cox regression analysis. Cluster analysis of 200 patients identified four homogenous phenotypes. Cluster C1 included patients with mild to moderate risk pulmonary arterial hypertension (PAH) with limited or no interstitial lung disease (ILD) and low DLCO with a 3-year survival of 81.5% (95% CI: 71.4–88.2). C2 had pre-capillary PH due to extensive ILD and worse 3-year survival compared to C1 (adjusted hazard ratio [HR] 3.14; 95% CI 1.66–5.94; p = 0.0004). C3 had severe PAH and a trend towards worse survival (HR 2.53; 95% CI 0.99–6.49; p = 0.052). Cluster C4 and C1 were similar with no difference in survival (HR 0.65; 95% CI 0.19–2.27, p = 0.507) but with a higher DLCO in C4. PH in SSc can be characterized into distinct clusters that differ in prognosis.</p></div

Baseline characteristics.

<p>Baseline characteristics.</p