The acute effects of prostacyclin on pulmonary hemodynamics in patients with pulmonary hypertension secondary to systemic sclerosis.
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.
Menon
N
N
Glasgow Royal Infirmary University National Health Service Trust, Scotland, UK.
McAlpine
L
L
Peacock
A J
AJ
Madhok
R
R
D000328:Adult; D011464:Epoprostenol / Q000627:therapeutic use*; D005260:Female; D006439:Hemodynamics / Q000187:drug effects; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology* / Q000503:physiopathology; D007275:Injections, Intravenous; D008875:Middle Aged; D011652:Pulmonary Circulation / Q000187:drug effects*; D012595:Scleroderma, Systemic / Q000150:complications*; D014665:Vasodilator Agents / Q000008:administration & dosage / Q000627:therapeutic use*
D014665:Vasodilator Agents; D011464:Epoprostenol