The acute effects of prostacyclin on pulmonary hemodynamics in patients with pulmonary hypertension secondary to systemic sclerosis

Abstract

Objective: To determine whether patients with isolated pulmonary hypertension secondary to systemic sclerosis (SSc) have a pulmonary vasculature that is responsive to treatment with vasodilators.

Methods: Seven SSc patients with pulmonary hypertension underwent right heart catheterization. Pulmonary pressures and cardiac output were measured before and during a systemic intravenous infusion of prostacyclin.

Results: Both pulmonary arterial mean pressure and pulmonary vascular resistance decreased significantly with prostacyclin infusion (median decrease 11% and 32%, respectively), with a concomitant increase in cardiac output (median increase 26%). No adverse hemodynamic effects were noted during the infusion.

Conclusion: Vasospasm contributes to the pulmonary hypertension complicating SSc. This feature may be amenable to treatment with vasodilators.