Background: The objective of this study was to investigate the prognostic impact of right ventricular (RV) function at rest and during exercise on survival in patients with systemic sclerosis (SSc) presenting for a screening of PH.

Methods: In this study, we analyzed data from SSc patients who underwent routinely performed examinations for PH screening including echocardiography and right heart catheterization at rest and during exercise. Uni- (Kaplan-Meier) and multivariable (Cox Regression) analyses were performed to identify prognostic parameters in SSc.

Results: Out of 285 SSc patients screened for PH a total of 225 patients (81.3% female, mean age 58.1±13.0 years, 68% limited cutaneous SSc, WHO-FC II-III 74%, 24 manifest PH) were included in the analysis (60 excluded due to missing key hemodynamic data). During a follow-up of 3.2±2.7 (median 2.6) years, 21 patients died or received lung transplantation. Tricuspid annular plane systolic excursion ≤18 mm (p=0.0004), increase of cardiac index during exercise (ΔCI) ≤2 l/min (p=0.0002), ventricular output reserve >3 mmHg/l/min (p=0.001), peak CI ≤5.5 l/min (p=0.01), pulmonary arterial compliance >2 ml/mmHg (p=0.0005) and RV function assessed by echocardiography at rest (p<0.0001) significantly predicted survival. In the multivariable analysis, RV function assessed by echocardiography at rest and diffusion capacity for carbon monoxide <65% were identified as independent prognostic predictors.

Conclusion: This study demonstrates that in addition to known prognostic predictors, assessment of right ventricular function may provide crucial information to identify SSc patients who are at a high risk of a reduced survival.