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Background: early detection of patients with scleroderma at risk for pulmonary hypertension (PH) could lead to a more timely intervention. The change in pulmonary artery systolic pressure (PASP) with exercise provides a possible tool for such detection. However, the mechanisms responsible for such PASP elevation (diastolic dysfunction versus pulmonary arteriopathy) are still a matter of debate.Objectives: we sought to determine the incidence of exercise-induced PH in a referral population of patients with scleroderma, and to examine the relation between exercise-induced PH and tissue-Doppler (TDI) derived left atrial pressure (LAP).Methods: thirty-three patients with scleroderma (4 men and 29 women; mean age 57.6 ± 9.3 years) and normal resting left ventricular (LV) function were studied. Five patients were excluded due to abnormal resting PASP (= 40 mmHg) and seven (21.1%) due to inexistent tricuspid regurgitation. All patients underwent exercise Doppler echocardiography using a standard cycloergometer protocol. Tricuspid regurgitation velocity, mitral E wave velocity, LV outflow tract time-velocity integral and LV septal E' wave were measured before and in peak exercise to derivate values for cardiac output (CO), LAP and pulmonary vascular resistance (PVR).Results: mean age of diagnosis was 49.1 ± 10.7 years, with 19.6% of patients having diffuse cutaneous systemic sclerosis; the mean DLCO was 90.1 ± 20.5%. With a mean workload of 68 ± 25 Watts, 18 of 21 (85.7%) patients demonstrated an increase in PASP to > 35 mm Hg plus an estimated right atrial pressure of 5 mmHg (from 27.1 ± 4.9 vs. 49.5 ± 10.9 mmHg, p = 0.042). The mean heart rates increased from 70.6 ± 14.1 mmHg to 114.7 ± 22.9 mmHg, p < 0.001. This was accompanied by a significant increase in PVR (0.4 ± 1.1 vs. 2.4 ± 0.8 Wood units, p < 0.001) and CO (4.4 ± 0.9 vs. 9.6 ± 1.8 l.min-1, p < 0.001) and by a slight drop in mean estimated LAP (14.9 ± 4.3 vs. 11.8 ± 3.4 mmHg, p = 0.007). Mean E wave/A wave ratio did not change during exercise (1.1 ± 0.26 vs. 1.3 ± 0.36, p = 0.131).Conclusion: exercise-induced PH is very common in patients with scleroderma, even when resting PASP is normal. Exercise Doppler echocardiography identifies scleroderma patients with an abnormal rise in PASP during exertion. The elevation of PASP is apparently related to an elevation of estimated PVR and not to a concomitant elevation in estimated LAP. Further studies are warranted in this field, to determine the prognostic implications of these findings.