IMPROVEMENT IN PULMONARY-HYPERTENSION AND HYPOXEMIA DURING NITRIC-OXIDE INHALATION IN A PATIENT WITH END-STAGE PULMONARY FIBROSIS

A patient with severe interstitial pulmonary fibrosis, hypoxemia, pulm onary hypertension, and cor pulmonale was given inhaled nitric oxide ( NO) followed by intravenous PGE(1) to assess the reversibility of pulm onary hypertension. During NO inhalation, there was marked reduction i n pulmonary vascular resistance, increased cardiac output, and dramati c improvement in arterial oxygenation. There was no effect on systemic vascular resistance. In contrast, intravenous PGE(1) led to rapid art erial oxygen desaturation and worsened dyspnea. The beneficial respons es to inhaled NO in this patient suggest that, even in severe chronic lung disease, reversible pulmonary vasoconstriction is present. Inhale d NO thus has a potential therapeutic role as a selective pulmonary va sodilator in patients with interstitial pulmonary fibrosis and cor pul monale.