Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis

Rheumatology (Oxford). 2004 Apr;43(4):461-6. doi: 10.1093/rheumatology/keh067. Epub 2004 Jan 6.


Objective: A prospective study to evaluate echocardiography and gas transfer (DLCO) by comparison with cardiac catheterization in discriminating between patients with and without systemic sclerosis-associated pulmonary arterial hypertension (SScPAH).

Method: A total of 137 (52 with and 85 without pulmonary fibrosis) had echocardiography and lung function tests within 3 months of their definitive invasive study.

Results: At cardiac catheterization 99 of these patients were found to have PAH, while PAH was excluded in 38. Echocardiographically estimated tricuspid gradient (TG) showed a moderate positive correlation (r(2) = 0.44, P<0.005) with both mean pulmonary pressure and invasively determined tricuspid gradient. DLCO showed a weak correlation (r(2 )= 0.09, P = 0.006), when compared with mean pulmonary arterial pressure. In total, 97% of patients with an echocardiographically determined TG of > 45 mmHg were found to have pulmonary hypertension at catheterization. However, no threshold could be defined with either screening test that safely excluded PAH.

Conclusions: The positive predictive accuracy of currently used non-invasive tests are adequate for the diagnosis of advanced PAH provided sufficiently high thresholds (TG > 45 mmHg or DLCO < 55% predicted) are used. These tests cannot be relied upon to exclude pulmonary hypertension where pre-test probability is high.

Publication types

  • Evaluation Study
  • Multicenter Study

MeSH terms

  • Aged
  • Blood Pressure
  • Carbon Monoxide
  • Cardiac Catheterization
  • Humans
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / diagnostic imaging
  • Hypertension, Pulmonary / etiology
  • Mass Screening / methods
  • Middle Aged
  • Prospective Studies
  • Pulmonary Artery / physiopathology
  • Pulmonary Diffusing Capacity
  • Pulmonary Fibrosis / complications
  • Scleroderma, Systemic / complications*
  • Ultrasonography


  • Carbon Monoxide