Echocardiography-Derived Exercise Pulmonary Hypertension and Longitudinal Changes in Pulmonary Artery Pressures in...

Pulmonary artery pressures (PAPs) often become abnormal during exercise in patients with systemic sclerosis (SSc). This study sought to determine whether exercise-induced pulmonary hypertension (EIPH) assessed by exercise stress echocardiography (ESE) would be associated with subsequent increases in resting PAPs in patients with SSc. Patients with SSc who underwent serial echocardiography > 6 months apart were retrospectively screened (n = 543). Of those, patients undergoing ESE were divided based on the presence of EIPH, defined as estimated mean PAP (mPAP) > 30 mmHg during exercise and a mPAP/cardiac output (CO) > 3 mmHg/L/min. Patients who did not undergo ESE and had a mPAP < 20 mmHg were selected as controls (n = 198). The primary endpoint was a longitudinal change in sPAP from baseline to last follow-up echocardiography. Compared to controls and patients with non-EIPH (n = 71), those with EIPH (n = 56) had a higher sPAP at baseline (22 ± 4 mmHg, 28 ± 6 mmHg, and 31 ± 6 mmHg, p < 0.0001). Over a median follow-up duration of 2.4 years, a longitudinal change in resting sPAP from baseline to follow-up echocardiography was higher in patients with EIPH than those without (+ 1.2 mmHg vs. -1.0 mmHg, p = 0.048). In a sensitivity analysis excluding 30 patients who received pulmonary vasodilators or heart failure medications after baseline echocardiography, the difference in sPAP change over time between the two groups increased further (+ 2.2 mmHg in the EIPH group vs. -1.6 mmHg in the non-EIPH group, p = 0.0008). In conclusion, echocardiography-derived EIPH was associated with a longitudinal increase in sPAP, supporting ESE as a non-invasive tool for risk stratification in SSc-PH.