RT Journal Article SR Electronic T1 Cardiopulmonary exercise testing for detecting pulmonary arterial hypertension in systemic sclerosis JF Heart JO Heart FD BMJ Publishing Group Ltd and British Cardiovascular Society SP 774 OP 782 DO 10.1136/heartjnl-2016-309981 VO 103 IS 10 A1 Daniel Dumitrescu A1 Christian Nagel A1 Gabor Kovacs A1 Tom Bollmann A1 Michael Halank A1 Jörg Winkler A1 Martin Hellmich A1 Ekkehard Grünig A1 Horst Olschewski A1 Ralf Ewert A1 Stephan Rosenkranz YR 2017 UL http://heart.bmj.com/content/103/10/774.abstract AB Objectives Pulmonary arterial hypertension (PAH) is a devastating disease with limited survival and occurs as a frequent complication in patients with systemic sclerosis (SSc). A definite diagnosis of PAH is obtained by right heart catheterisation (RHC); however, the initial suspicion is raised by non-invasive methods. We assessed the diagnostic accuracy of key parameters derived from cardiopulmonary exercise testing (CPET) for detecting and ruling out SSc-associated PAH.Methods In a multicentre setting, we prospectively evaluated 173 consecutive patients with SSc without known PAH, but with clinical suspicion of PAH. Each patient underwent CPET and RHC.Results RHC identified PAH in 48 patients (27.8%), postcapillary pulmonary hypertension (PH) in 10 patients (5.8%) and ruled out PH in 115 patients (66.5%). CPET parameters correlated significantly with pulmonary haemodynamics. PeakVO2 and VE/VCO2 showed highest correlations with pulmonary arterial pressure, transpulmonary pressure gradient and pulmonary vascular resistance. Several parameters showed high sensitivity and specificity for PAH detection by receiver operating characteristic analysis. However, peakVO2 showed highest diagnostic accuracy (sensitivity 87.5%, specificity 74.8% at a threshold level of 13.8 mL/min/kg). A peakVO2 of >18.7 mL/kg/min was reached by 38/173 patients (22%) and excluded PAH in our cohort (negative predictive value 1.0). A nadir VE/VCO2 ratio of >45.5 showed a positive predictive value of 1.0. Diagnostic accuracy was highest in patients with low pulmonary arterial wedge pressure (<12 mm Hg). There were no study-related serious adverse events.Conclusions CPET is a safe and valuable method in the non-invasive detection of SSc-associated PAH. It may be particularly beneficial for reducing unnecessary RHC procedures.