Hemodynamic effects of epoprostenol in patients with systemic sclerosis and pulmonary hypertension.
OBJECTIVE
To determine the cause of pulmonary hypertension (PH) in systemic sclerosis (SSc) patients since PH can occur because of pulmonary arteriopathy, pulmonary parenchymal destruction, and left ventricular cardiac dysfunction.


METHODS
Consecutive case series in a university hospital.


METHODS
Nine SSc patients with PH (mean pulmonary artery pressure, 41 mm Hg), with (n = 6) or without (n = 3) concomitant interstitial lung disease (ILD).


METHODS
Acute infusion of epoprostenol was begun at 2 ng/kg/min and was titrated upward at a rate of 2 ng/kg/min every 30 min until symptomatic complications developed or pulmonary artery vascular resistance (PVR) was reduced by 50%.


RESULTS
Eight of nine patients demonstrated a reduction of > or = 20% in PVR, suggesting that vasoreactivity is common despite the presence of significant ILD. A single patient had no response to infusion with unchanged hemodynamics and oxygenation. One patient developed hypoxemia as cardiac output increased, suggesting a worsening of ventilation/perfusion matching or the presence of an anatomic shunt. Acute pulmonary edema developed in one patient at an infusion rate of 6 ng/kg/min. The results of cardiac catheterization suggested that pulmonary edema was caused by SSc heart disease.


CONCLUSIONS
SSc patients with ILD have diverse and sometimes multiple causes of PH that can be determined by short-term epoprostenol infusion. Beneficial effects can be obtained from epoprostenol despite extensive ILD.
Strange
C
C
Division of Pulmonary and Critical Care Medicine, Allergy and Clinical Immunology, Medical University of South Carolina, Charleston, USA. strangec@musc.edu
Bolster
M
M
Mazur
J
J
Taylor
M
M
Gossage
J R
JR
Silver
R
R
D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage*; D006328:Cardiac Catheterization; D002302:Cardiac Output / Q000187:drug effects; D015150:Echocardiography, Doppler; D011464:Epoprostenol / Q000008:administration & dosage*; D005260:Female; D006439:Hemodynamics / Q000187:drug effects*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology*; D007262:Infusions, Intravenous; D008297:Male; D008875:Middle Aged; D011669:Pulmonary Wedge Pressure / Q000187:drug effects; D012595:Scleroderma, Systemic / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000503:physiopathology*; D014109:Total Lung Capacity
D000959:Antihypertensive Agents; D011464:Epoprostenol