Improvement in pulmonary hypertension and hypoxemia during nitric oxide inhalation in a patient with end-stage pulmonary fibrosis

Am J Respir Crit Care Med. 1994 Mar;149(3 Pt 1):811-4. doi: 10.1164/ajrccm.149.3.8118653.


A patient with severe interstitial pulmonary fibrosis, hypoxemia, pulmonary hypertension, and cor pulmonale was given inhaled nitric oxide (NO) followed by intravenous PGE1 to assess the reversibility of pulmonary hypertension. During NO inhalation, there was marked reduction in pulmonary vascular resistance, increased cardiac output, and dramatic improvement in arterial oxygenation. There was no effect on systemic vascular resistance. In contrast, intravenous PGE1 led to rapid arterial oxygen desaturation and worsened dyspnea. The beneficial responses to inhaled NO in this patient suggest that, even in severe chronic lung disease, reversible pulmonary vasoconstriction is present. Inhaled NO thus has a potential therapeutic role as a selective pulmonary vasodilator in patients with interstitial pulmonary fibrosis and cor pulmonale.

Publication types

  • Case Reports

MeSH terms

  • Administration, Inhalation
  • Adult
  • Alprostadil / pharmacology
  • Alprostadil / therapeutic use*
  • Blood Gas Analysis
  • Drug Therapy, Combination
  • Hemodynamics / drug effects*
  • Humans
  • Hypertension, Pulmonary / blood
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / physiopathology
  • Hypoxia / blood
  • Hypoxia / drug therapy*
  • Hypoxia / etiology
  • Hypoxia / physiopathology
  • Infusions, Intravenous
  • Male
  • Nitric Oxide / pharmacology
  • Nitric Oxide / therapeutic use*
  • Pulmonary Fibrosis / complications*
  • Pulmonary Fibrosis / diagnostic imaging
  • Pulmonary Fibrosis / pathology
  • Pulmonary Gas Exchange / drug effects
  • Tomography, X-Ray Computed


  • Nitric Oxide
  • Alprostadil