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