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Cardiopulmonary exercise testing for detecting pulmonary arterial hypertension in systemic sclerosis
  1. Daniel Dumitrescu1,2,
  2. Christian Nagel3,4,
  3. Gabor Kovacs5,
  4. Tom Bollmann6,
  5. Michael Halank7,
  6. Jörg Winkler8,
  7. Martin Hellmich9,
  8. Ekkehard Grünig3,
  9. Horst Olschewski5,
  10. Ralf Ewert6,
  11. Stephan Rosenkranz1,2
  1. 1Herzzentrum der Universität zu Köln, Klinik III für Innere Medizin, Cologne, Germany
  2. 2Cologne Cardiovascular Research Center (CCRC), University of Cologne, Germany
  3. 3Center for Pulmonary Hypertension, Thoraxklinik Heidelberg, Heidelberg, Germany
  4. 4Lung Center, Klinikum Mittelbaden, Baden-Baden Balg, Baden-Baden, Germany
  5. 5Division of Pulmonology, Department of Internal Medicine, Ludwig Boltzmann Institute for Lung Vascular Research, and Medical University of Graz, Graz, Austria
  6. 6Department of Internal Medicine B—Cardiology, Intensive Care, Pulmonary Medicine and Infectious Diseases, University of Greifswald, Greifswald, Germany
  7. 7Department of Internal Medicine I, Carl Gustav Carus University Hospital, Technical University of Dresden, Dresden, Germany
  8. 8Klinik für Innere Medizin I, University of Leipzig, Leipzig, Germany
  9. 9Universitätsklinikum Köln, Institut für Medizinische Statistik, Cologne, Germany
  1. Correspondence to Dr Stephan Rosenkranz, Klinik III für Innere Medizin, Cologne Cardiovascular Research Center (CCRC), Herzzentrum der Universität zu Köln, Kerpener Str. 62, 50937 Cologne, Germany; stephan.rosenkranz{at}uk-koeln.de

Abstract

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.

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Footnotes

  • DD and CN contributed equally to this study.

  • Contributors All coauthors participated in aspects of the study that qualifies them as authors: DD contributed to the study conception, study design, patient data, analysis/interpretation of the data, statistical analysis and writing the final version of the article. CN contributed to the study conception, study design, patient data, analysis/interpretation of the data, statistical analysis and writing the final version of the article. GK, TB, MH (Dresden), JW, EG and HO contributed to the study conception, patient data, analysis of the data and critical review of article. MH (Cologne) contributed to the study conception, study design, statistical analysis and critical review of the article. RE contributed to the study conception, study design, patient data, analysis/interpretation of the data and critical review of article. SR contributed to the study conception, study design, patient data, analysis/interpretation of the data, statistical analysis and writing the final version of the article. DD and CN (the first two authors) contributed equally to this manuscript, so that a shared first authorship is considered appropriate.

  • Funding This work was supported in part by a research grant from Actelion Pharmaceuticals (DD, SR).

  • Competing interests None.

  • Ethics approval Local institutional review board.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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