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

Charlie Strange, Marcy Bolster, Joe Mazur, Marian Taylor, James R. Gossage, Richard Silver

Research output: Contribution to journalArticlepeer-review

38 Scopus citations


Study objectives: To determine the cause of pulmonary hypertension (PH) in systemic sclerosis (SSc) patients since PH can occur because of pulmonary arteriopathy, pulmonary parenchymal destruction, and left ventricular cardiac 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 titrated upward at a rate of 2 ng/kg/min every 30 min until symptomatic complications developed or pulmonary artery vascular resistance (PVR) was reduced by 50%. Results: Eight of nine patients demonstrated a reduction of ≥ 20% in PVR, suggesting that vasoreactivity is common despite the presence of significant ILD. A single patient had no response to infusion with unchanged hemodynamics and oxygenation. One patient developed hypoxemia as cardiac output increased, suggesting a worsening of ventilation/perfusion matching or the presence of an anatomic shunt. Acute pulmonary edema developed in one patient at an infusion rate of 6 ng/kg/min. The results of cardiac catheterization suggested that pulmonary edema was caused by SSc heart disease. Conclusion: SSc patients with ILD have diverse and sometimes multiple causes of PH that can be determined by short-term epoprostenol infusion. Beneficial effects can be obtained from epoprostenol despite extensive ILD.

Original languageEnglish (US)
Pages (from-to)1077-1082
Number of pages6
Issue number4
StatePublished - 2000


  • Epoprostenol
  • Interstitial lung disease
  • Pulmonary hypertension
  • Systemic sclerosis

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine
  • Cardiology and Cardiovascular Medicine


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