title: Cardiopulmonary exercise testing for detecting pulmonary arterial hypertension in systemic sclerosis.
abstract: 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.
  
  
  
  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.
  
  
  
  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. PeakVO2and VE/VCO2showed 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, peakVO2showed highest diagnostic accuracy (sensitivity 87.5%, specificity 74.8% at a threshold level of 13.8 mL/min/kg). A peakVO2of >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/VCO2ratio 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.
  
  
  
  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.
authors.affiliation: Herzzentrum der Universität zu Köln, Klinik III für Innere Medizin, Cologne, Germany.|Cologne Cardiovascular Research Center (CCRC), University of Cologne, Germany.
authors.forename: Daniel
authors.initials: D
authors.lastname: Dumitrescu
authors.affiliation: Center for Pulmonary Hypertension, Thoraxklinik Heidelberg, Heidelberg, Germany.|Lung Center, Klinikum Mittelbaden, Baden-Baden Balg, Baden-Baden, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Nagel
authors.affiliation: Division of Pulmonology, Department of Internal Medicine, Ludwig Boltzmann Institute for Lung Vascular Research, and Medical University of Graz, Graz, Austria.
authors.forename: Gabor
authors.initials: G
authors.lastname: Kovacs
authors.affiliation: Department of Internal Medicine B-Cardiology, Intensive Care, Pulmonary Medicine and Infectious Diseases, University of Greifswald, Greifswald, Germany.
authors.forename: Tom
authors.initials: T
authors.lastname: Bollmann
authors.affiliation: Department of Internal Medicine I, Carl Gustav Carus University Hospital, Technical University of Dresden, Dresden, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Halank
authors.affiliation: Klinik für Innere Medizin I, University of Leipzig, Leipzig, Germany.
authors.forename: Jörg
authors.initials: J
authors.lastname: Winkler
authors.affiliation: Universitätsklinikum Köln, Institut für Medizinische Statistik, Cologne, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Hellmich
authors.affiliation: Center for Pulmonary Hypertension, Thoraxklinik Heidelberg, Heidelberg, Germany.
authors.forename: Ekkehard
authors.initials: E
authors.lastname: Grünig
authors.affiliation: Division of Pulmonology, Department of Internal Medicine, Ludwig Boltzmann Institute for Lung Vascular Research, and Medical University of Graz, Graz, Austria.
authors.forename: Horst
authors.initials: H
authors.lastname: Olschewski
authors.affiliation: Department of Internal Medicine B-Cardiology, Intensive Care, Pulmonary Medicine and Infectious Diseases, University of Greifswald, Greifswald, Germany.
authors.forename: Ralf
authors.initials: R
authors.lastname: Ewert
authors.affiliation: Herzzentrum der Universität zu Köln, Klinik III für Innere Medizin, Cologne, Germany.|Cologne Cardiovascular Research Center (CCRC), University of Cologne, Germany.
authors.forename: Stephan
authors.initials: S
authors.lastname: Rosenkranz
mesh_terms: D000368:Aged; D000465:Algorithms; D006328:Cardiac Catheterization / Q000379:methods; D004452:Echocardiography; D005080:Exercise Test / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012372:ROC Curve; D015203:Reproducibility of Results; D012595:Scleroderma, Systemic / Q000150:complications* / Q000175:diagnosis; D014655:Vascular Resistance / Q000502:physiology*; D016278:Ventricular Function, Right / Q000502:physiology