Objectives .– Resting pulmonary hypertension (PH) is a powerful determinant of poor outcome in patients with systemic scleroderma (SSc). In various cardiac diseases, exercise-induced PH (EIPH) is considered as an early stage of resting PH, and may predict the occurrence of adverse events. We hypothesized that the presence of EIPH is a predictor of the onset of resting PH in patients with SSC.

Patients and methods .– Thirty-three patients with diagnosis of SSc disease were prospectively submitted to comprehensive both resting and exercise stress echocardiography between January 2009 and November 2012.

Resting PH was defined as a transtricuspid pressure gradient (TTG) ≥ 30 mmHg, and EIPH as TTG ≥ 40 mmHg Patients were prospectively follow-up and regularly performed resting echocardiography.

Results .– There was a significant increase in TTG from rest to exercise (21.5 ± 7.7 vs. 37.5 ± 15.2, P < 0.001). There were two patients with baseline resting PH (6%) and 15 patients with EIPH (45%). Of note, all patients with resting PH have developed EIPH. Mean follow-up was 25 ± 13 months (from 1 to 52) and six patients have experienced resting PH during this period, resulting in a mean time interval between baseline echocardiography and occurrence of resting PH of 8 ± 7 months (from 1 to 18). Of interest, all patients who developed resting PH during the follow-up have baseline EIPH (60%). In contrast, none of patients without baseline EIPH developed resting PH during the follow-up (0%). Although patients who developed resting PH had significant higher baseline resting TTG (26.6 ± 4.0 vs. 17.8 ± 5.7, P = 0.03), the difference in baseline exercise TTG was markedly higher (53.7 ± 14.0 vs. 31.4 ± 11.9, P = 0.001). Using logistic regression, after adjustment for age, baseline EIPH was independently associated with the occurrence of resting PH during the follow-up ( P = 0.0014).

Conclusion .– EIPH may be frequent in SSc patients and could be a predictive factor of rapid and early onset of resting PH. Exercise stress echocardiography may be useful to identify subset of patients at high risk to developed resting PH and thus, could benefit from more aggressive therapeutic strategy. Nevertheless, further data in larger cohort are needed to confirm our results.