Impact of pulmonary fibrosis and elevated pulmonary pressures on right ventricular function in patients with systemic sclerosis

Rheumatology (Oxford). 2016 Mar;55(3):504-12. doi: 10.1093/rheumatology/kev342. Epub 2015 Oct 15.


Objectives: Right ventricular (RV) dysfunction is of great prognostic value in patients with SSc. The aim of the present study was to assess in these patients the relationship between pulmonary fibrosis and elevated pulmonary pressure (PHT) with RV function.

Methods: A total of 102 SSc patients who underwent thoracic CT and transthoracic echocardiography were included. Speckle tracking-derived RV free wall strain was used to assess RV function.

Results: A total of 51 (50%) SSc patients did not have pulmonary fibrosis or PHT, 32 (31%) patients had pulmonary fibrosis but no PHT and the remaining 19 (19%) patients had both pulmonary fibrosis and PHT. Patients with both pulmonary fibrosis and PHT had the most impaired RV free wall strain [-16.8% (s.d. 3.1)] compared with patients with pulmonary fibrosis and no PHT [-21.5% (s.d. 3.6)] and patients with no pulmonary fibrosis and no PHT [-24.0% (s.d. 4.4)]. All three SSc groups showed impaired RV free wall strain compared with controls [-28.0% (s.d. 4.2)]. Importantly, multivariate regression analysis demonstrated that pulmonary fibrosis and left ventricular ejection fraction were independently associated with impaired RV free wall strain in SSc patients.

Conclusion: SSc patients show impaired RV function compared with controls. Both pulmonary fibrosis and PHT are independently associated with RV dysfunction.

Keywords: pulmonary fibrosis; pulmonary hypertension; right ventricular function; systemic sclerosis.

Publication types

  • Comparative Study

MeSH terms

  • Adult
  • Age Factors
  • Aged
  • Analysis of Variance
  • Case-Control Studies
  • Echocardiography / methods
  • Female
  • Humans
  • Hypertension, Pulmonary / complications*
  • Hypertension, Pulmonary / diagnosis
  • Image Interpretation, Computer-Assisted*
  • Linear Models
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Pulmonary Fibrosis / complications*
  • Pulmonary Fibrosis / diagnostic imaging
  • Retrospective Studies
  • Risk Assessment
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / diagnosis
  • Severity of Illness Index
  • Sex Factors
  • Tomography, X-Ray Computed / methods
  • Ventricular Dysfunction, Right / diagnostic imaging*
  • Ventricular Dysfunction, Right / etiology*
  • Ventricular Dysfunction, Right / physiopathology