Echocardiography-Derived Exercise Pulmonary Hypertension and Longitudinal Changes in Pulmonary Artery Pressures in Systemic Sclerosis: A Non-Invasive Assessment for Risk Stratification

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.