Hemodynamic effects of epoprostenol in patients with systemic sclerosis and pulmonary hypertension

Study objectives: To determine the cause of pulmonary hypertension (PH) in systemic sclerosis (SSc) patients since PH can occur because of pulmonary a rteriopathy, pulmonary parenchymal destruction, and left ventricular cardia c dysfunction, Design and setting: Consecutive case series in a university hospital. Patients: Nine SSc patients with PH (mean pulmonary artery pressure, 41 mm Hg), with (n = 6) or without (n = 3) concomitant interstitial lung disease (ILD), Methods: Acute infusion of epoprostenol was begun at 2 ng/kg/min and was ti trated upward at a rate of 2 ng/kg/min every 30 min until symptomatic compl ications developed or pulmonary artery vascular resistance (PVR) was reduce d by 50%, Results: Eight of nine patients demonstrated a reduction of greater than or equal to 20% in PVR, suggesting that vasoreactivity is common despite the presence of significant ILD. A single patient had no response to infusion w ith unchanged hemodynamics and oxygenation, One patient developed hypoxemia as cardiac output increased, suggesting a worsening of ventilation/perfusi on matching or the presence of an anatomic shunt. Acute pulmonary edema dev eloped in one patient at an infusion rate of 6 ng/kg/min. The results of ca rdiac catheterization suggested that pulmonary edema was caused by SSc hear t disease. Conclusion: SSc patients with ILD have diverse and sometimes multiple cause s of PH that can be determined by short-term epoprostenol infusion. Benefic ial effects can be obtained from epoprostenol despite extensive ILD.