Exercise-Doppler echocardiography in systemic sclerosis: a useful tool to track serial changes in pulmonary artery systolic pressure.

Purpose: Patients with systemic sclerosis (SSc) are at risk of developing pulmonary arterial hypertension (PAH), which is associated with a poor prognosis. Exercise- Doppler echocardiography (EDE) may enable identification of exercise-induced increase in pulmonary arterial systolic pressure (PASP). The aim of our study was to evaluate whether exercise-induced PASP increase may predict resting PASP increase. Methods: We selected 37 SSc patients (age = 58+13 ys, 82% females, 74% limited cutaneous form) with normal resting PASP (, 40 mmHg), who had developed exercise-induced PASP increase at a previous graded bicycle semi-supine EDE. They underwent the same examination after at least six months. Patients discovered with a resting PASP . 40 mmHg did not perform the exercise. A cut-off value of PASP . 40 mmHg was considered a significant resting PASP increase, whereas PASP >= 50 mmHg at peak stress was considered a significant exercise-inducedincrease. No patient had significant pulmonary fibrosis at chest computed tomography or chest X-ray. Results: Mean follow-up time between the two EDE was 21 + 12 months, during which two deaths occurred. Seven patients (19%) developed resting PASP increase (mean time follow-up 22 + 15 months), 24 (65%) showed the same behaviour as previous examination, 2 (7%) had died, and 4 (11%) did not confirm the exercise-induced PASP increase, with 3 patients having started Calcium-antagonist therapy. Conclusion: EDE is a feasible test to track serial changes over time in PASP in patients at high risk of developing PAH. Exercise-induced PASP increase is frequently associated with poor outcome or development of overt resting PASP increase.