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Inappropriate exercise-induced increase in pulmonary artery pressure in patients with systemic sclerosis
  1. Michele D'Alto1,
  2. Stefano Ghio2,
  3. Antonello D'Andrea1,
  4. Anna Sara Pazzano2,
  5. Paola Argiento1,
  6. Rita Camporotondo2,
  7. Francesca Allocca1,
  8. Laura Scelsi2,
  9. Giovanna Cuomo3,
  10. Roberto Caporali4,
  11. Lorenzo Cavagna4,
  12. Gabriele Valentini3,
  13. Raffaele Calabrò1
  1. 1Department of Cardiology, Second University of Naples – Monaldi Hospital, Naples, Italy
  2. 2Department of Cardiology, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy
  3. 3Unit of Rheumatology, Second University of Naples, Italy
  4. 4Unit of Rheumatology, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy
  1. Correspondence to Michele D'Alto, Department of Cardiology, Second University of Naples – Monaldi Hospital, Via L. Bianchi, Naples 80131, Italy; mic.dalto{at}


Background Recent data show that there is an unexpectedly high prevalence of ‘inappropriate’ pulmonary responses to exercise among patients with systemic sclerosis (SS). However, no consensus exists as to which threshold of pulmonary artery systolic pressure (PASP) can be considered diagnostically relevant.

Aim To evaluate pulmonary vascular reserve and right ventricular function changes induced by exercise in SS patients without overt pulmonary arterial hypertension.

Methods and results The study enrolled 172 consecutive SS patients in NYHA class I–II, with a peak tricuspid regurgitant jet velocity at echocardiography not greater than 3 m/s, and 88 control subjects. Echocardiography was performed at rest and at the end of a maximal exercise test. SS patients showed a higher exercise-induced PASP than control subjects (36.9±8.7 vs 25.9±3.3 mm Hg, p=0.00008). The response to effort was higher in the presence of moderate interstitial lung disease (39.7±9.3 vs 36.0±8.4 mm Hg, p=0.016) or subclinical left ventricular diastolic dysfunction (42.3±5.8 vs 37.0±8.6 mm Hg, p=0.015). In control subjects, PASP values were normally distributed at rest and after exercise. In SS patients, the distribution was normal at rest but bimodal after exercise, with a second peak at 52.2 mm Hg including 13% of the total SS population. Patients in this subgroup showed subtle abnormalities of right ventricular function at rest and, most importantly, a blunted increase in right ventricular systolic function with exercise.

Conclusion Exercise echocardiography may identify a subset of SS patients with an inappropriate exercise-induced increase in PASP and early signs of right ventricular dysfunction.

  • Systemic sclerosis
  • stress echocardiography
  • right ventricular function
  • echocardiography-exercise
  • pulmonary vascular disease

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  • See Editorial, p 94

  • MD'A and SG contributed equally in planning the study and in writing the manuscript.

  • Competing interests None.

  • Ethics approval This study was conducted with the approval of the ethics committees of the Second University of Naples and Fondazione IRCCS Policlinico S. Matteo of Pavia, Italy.

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

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