Inhaled prostacyclin and iloprost in severe pulmonary hypertension secondary to lung fibrosis

Am J Respir Crit Care Med. 1999 Aug;160(2):600-7. doi: 10.1164/ajrccm.160.2.9810008.


Pulmonary hypertension is a life-threatening complication of lung fibrosis. Vasodilator therapy is difficult owing to systemic side effects and pulmonary ventilation-perfusion mismatch. We compared the effects of intravenous prostacyclin and inhaled NO and aerosolized prostacyclin in randomized order and, in addition, tested for effects of oxygen and systemic calcium antagonists (CAAs) in eight patients with lung fibrosis and pulmonary hypertension. Aerosolized prostaglandin (PG)I(2) caused preferential pulmonary vasodilatation with a decrease in mean pulmonary arterial pressure from 44.1 +/- 4.2 to 31.6 +/- 3.1 mm Hg, and pulmonary vascular resistance (RL) from 810 +/- 226 to 386 +/- 69 dyn. s. cm(-)(5) (p < 0.05, respectively). Systemic arterial pressure, arterial oxygen saturation, and pulmonary right-to-left shunt flow, measured by multiple inert gas analysis, were not significantly changed. Inhaled NO similarly resulted in selective pulmonary vasodilatation, with RL decreasing from 726 +/- 217 to 458 +/- 81 dyn. s. cm(-)(5). In contrast, both intravenous PGI(2) and CAAs were not pulmonary selective, resulting in a significant drop in arterial pressure. In addition, PGI(2) infusion caused a marked increase in shunt flow. Long-term therapy with aerosolized iloprost (long-acting PGI(2) analog) resulted in unequivocal clinical improvement from a state of immobilization and severe resting dyspnea in a patient with decompensated right heart failure. We concluded that, in pulmonary hypertension secondary to lung fibrosis, aerosolization of PGI(2) or iloprost causes marked pulmonary vasodilatation with maintenance of gas exchange and systemic arterial pressure. Long-term therapy with inhaled iloprost may be life saving in decompensated right heart failure from pulmonary hypertension secondary to lung fibrosis.

Publication types

  • Clinical Trial
  • Comparative Study
  • Randomized Controlled Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Administration, Inhalation
  • Adult
  • Aged
  • Atrial Function, Right / drug effects
  • Calcium Channel Blockers / administration & dosage
  • Calcium Channel Blockers / adverse effects
  • Drug Therapy, Combination
  • Epoprostenol / administration & dosage*
  • Epoprostenol / adverse effects
  • Female
  • Heart Failure / drug therapy
  • Heart Failure / etiology
  • Humans
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / etiology
  • Iloprost / administration & dosage*
  • Iloprost / adverse effects
  • Infusions, Intravenous
  • Lung / blood supply
  • Male
  • Middle Aged
  • Nifedipine / administration & dosage
  • Nifedipine / adverse effects
  • Nitric Oxide / administration & dosage
  • Nitric Oxide / adverse effects
  • Pulmonary Fibrosis / complications*
  • Pulmonary Fibrosis / drug therapy
  • Pulmonary Gas Exchange / drug effects
  • Pulmonary Wedge Pressure / drug effects
  • Vascular Resistance / drug effects
  • Vasodilator Agents / administration & dosage*
  • Vasodilator Agents / adverse effects
  • Ventilation-Perfusion Ratio / drug effects
  • Vital Capacity / drug effects


  • Calcium Channel Blockers
  • Vasodilator Agents
  • Nitric Oxide
  • Epoprostenol
  • Nifedipine
  • Iloprost