title: Stress Doppler echocardiography for early detection of systemic sclerosis-associated pulmonary arterial hypertension.
abstract: BACKGROUND
  In patients with systemic sclerosis (SSc), associated pulmonary arterial hypertension (SSc-APAH) is the leading cause of death. The objective of this prospective screening study was to analyse sensitivity and specificity of stress Doppler echocardiography (SDE) in detecting pulmonary hypertension (PH).
  
  
  METHODS
  Pulmonary artery pressures and further parameters of PH were assessed by echocardiography and right heart catheterisation (RHC) at rest and during exercise in patients with SSc. Investigators of RHC were blinded to the results of non-invasive measurements.
  
  
  RESULTS
  Of 76 patients with SSc (64 were female and mean age was 58±14 years), 22 (29 %) had manifest PH confirmed by RHC: four had concomitant left heart diseases, three had lung diseases, and 15 had SSc-APAH. Echocardiography at rest missed PH diagnosis in five of 22 patients with PH when a cutoff value for systolic pulmonary arterial pressure (PASP) was more than 40 mm Hg at rest. The sensitivity of echocardiography at rest was 72.7 % (95 % confidence interval (CI) 0.52-0.88), and specificity was 88.2 % (95 % CI 0.78-0.95). When a cutoff value for PASP was more than 45 mm Hg during low-dose exercise, SDE missed PH diagnosis in one of the 22 patients with PH and improved sensitivity to 95.2 % (95 % CI 0.81-1.0) but reduced specificity to 84.9 % (95 % CI 0.74-0.93). Reduction of specificity was partly due to concomitant left heart disease.
  
  
  CONCLUSIONS
  The results of this prospective cross-sectional study using RHC as gold standard in all patients showed that SDE markedly improved sensitivity in detecting manifest PH to 95.2 % compared with 72.7 % using echocardiography at rest only. Thus, for PH screening in patients with SSc, echocardiography should be performed at rest and during exercise.
  
  
  BACKGROUND
  ClinicalTrials.gov NCT01387035. Registered 29 June 2011.
authors.affiliation: Centre for Pulmonary Hypertension Thoraxclinic, University Hospital Heidelberg, Amalienstr. 5, 69126, Heidelberg, Germany. c.nagel@klinikum-mittelbaden.de.|Lung Centre, Klinikum Mittelbaden, Balger Str. 50, 76532, Baden-Baden Balg, Germany. c.nagel@klinikum-mittelbaden.de.
authors.forename: Christian
authors.initials: C
authors.lastname: Nagel
authors.affiliation: Centre for Pulmonary Hypertension Thoraxclinic, University Hospital Heidelberg, Amalienstr. 5, 69126, Heidelberg, Germany. philipphenn27@gmail.com.
authors.forename: Philipp
authors.initials: P
authors.lastname: Henn
authors.affiliation: Centre for Pulmonary Hypertension Thoraxclinic, University Hospital Heidelberg, Amalienstr. 5, 69126, Heidelberg, Germany. nicola.ehlken@med.uni-heidelberg.de.
authors.forename: Nicola
authors.initials: N
authors.lastname: Ehlken
authors.affiliation: Department of Cardiothoracic Sciences, Monaldi Hospital, Second University of Naples, Via Leonardo Bianchi, 1, 80131, Naples, Italy. antonellodandrea@libero.it.
authors.forename: Antonello
authors.initials: A
authors.lastname: D'Andrea
authors.affiliation: Division of Rheumatology, University Hospital Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany. norbert.blank@med.uni-heidelberg.de.
authors.forename: Norbert
authors.initials: N
authors.lastname: Blank
authors.affiliation: Department of Cardiology and Cardiac Surgery, University Hospital "Scuola Medica Salernitana", Via Pr. Amedeo, 36-83023 Lauro (AV), Salerno, Italy. ebossone@hotmail.com.
authors.forename: Eduardo
authors.initials: E
authors.lastname: Bossone
authors.affiliation: Rheumapraxis Landau, Industriestraße 9, 76829, Landau in der Pfalz, Germany. info@rheumapraxis-landau.de.
authors.forename: Anke
authors.initials: A
authors.lastname: Böttger
authors.affiliation: Department of Rheumatology, ACURA-Klinik Baden-Baden, Rotenbachtalstr. 5, 76530, Baden-Baden, Germany. c.fiehn@acura-kliniken.com.
authors.forename: Christoph
authors.initials: C
authors.lastname: Fiehn
authors.affiliation: Department of Human Genetics, University of Heidelberg, Im Neuenheimer Feld 366, 69120, Heidelberg, Germany. christine.fischer@med.uni-heidelberg.de.
authors.forename: Christine
authors.initials: C
authors.lastname: Fischer
authors.affiliation: Division of Rheumatology, University Hospital Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany. hannes.lorenz@med.uni-heidelberg.de.
authors.forename: Hanns-Martin
authors.initials: HM
authors.lastname: Lorenz
authors.affiliation: Klinikum Darmstadt, Medizinische Klinik III, Grafenstraße 9, 64283, Darmstadt, Germany. frank.stoeckl@klinikum-darmstadt.de.
authors.forename: Frank
authors.initials: F
authors.lastname: Stöckl
authors.affiliation: Centre for Pulmonary Hypertension Thoraxclinic, University Hospital Heidelberg, Amalienstr. 5, 69126, Heidelberg, Germany. ekkehard.gruenig@med.uni-heidelberg.de.
authors.forename: Ekkehard
authors.initials: E
authors.lastname: Grünig
authors.affiliation: Centre for Pulmonary Hypertension Thoraxclinic, University Hospital Heidelberg, Amalienstr. 5, 69126, Heidelberg, Germany. benjamin.egenlauf@med.uni-heidelberg.de.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Egenlauf
mesh_terms: D000328:Adult; D000368:Aged; D003430:Cross-Sectional Studies; D042241:Early Diagnosis; D015150:Echocardiography, Doppler / Q000379:methods; D025401:Echocardiography, Stress / Q000379:methods*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging* / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012595:Scleroderma, Systemic / Q000150:complications* / Q000000981:diagnostic imaging*