Background: Inhaled aerosolized iloprost, a stable prostacyclin analogue, h
as been considered a selective pulmonary vasodilator in the management of p
ulmonary hypertension.
Objective: To assess the efficacy of inhaled iloprost in the treatment of l
ife-threatening pulmonary hypertension.
Design: Open, uncontrolled, multicenter study.
Setting: Intensive care units and pulmonary hypertension clinics at six uni
versity hospitals in Germany.
Patients: 19 patients who had progressive right-heart failure despite recei
ving maximum conventional therapy (12 with primary pulmonary hypertension,
3 with pulmonary hypertension related to collagen vascular disease without
lung fibrosis, and 4 with secondary pulmonary hypertension).
Intervention: Inhaled iloprost, 6 to 12 times daily (50 to 200 mu g/d).
Measurements: Right-heart catheterization and distance walked in 6 minutes
at baseline and after 3 months of therapy.
Results: During the first 3 months of therapy, New York Heart Association f
unctional class improved in 8 patients and was unchanged in 7 patients. Fou
r patients died, 3 of right-heartfailure and 1 of sepsis. The acute hemodyn
amic response to inhaled iloprost was predominant pulmonary vasodilatation
with little systemic effect at baseline and at 3 months (data available for
12 patients). Hemodynamic variables were improved at 3 months, and the dis
tance walked in 6 minutes improved by 148 m (95% CI, 4.5 to 282 m; P = 0.04
8). Of the 15 patients who continued to use inhaled iloprost, 8 stopped: Fo
ur had lung transplantation, 1 switched to intravenous prostacyclin therapy
, and 3 died. Seven patients are still receiving inhaled iloprost (mean +/-
SD) duration of therapy, 536 +/- 309 days; mean dosage, 164 +/- 38 mu g/d).
Conclusions: Inhaled iloprost may offer a new therapeutic option for improv
ement of hemodynamics and physical function in patients with life-threateni
ng pulmonary hypertension and progressive right-heart failure that is refra
ctory to conventional therapy.