title: Inhaled iloprost to treat severe pulmonary hypertension. An uncontrolled trial. German PPH Study Group.
abstract: BACKGROUND
  Inhaled aerosolized iloprost, a stable prostacyclin analogue, has been considered a selective pulmonary vasodilator in the management of pulmonary hypertension.
  
  
  OBJECTIVE
  To assess the efficacy of inhaled iloprost in the treatment of life-threatening pulmonary hypertension.
  
  
  METHODS
  Open, uncontrolled, multicenter study.
  
  
  METHODS
  Intensive care units and pulmonary hypertension clinics at six university hospitals in Germany.
  
  
  METHODS
  19 patients who had progressive right-heart failure despite receiving 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).
  
  
  METHODS
  Inhaled iloprost, 6 to 12 times daily (50 to 200 microg/d).
  
  
  METHODS
  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 functional class improved in 8 patients and was unchanged in 7 patients. Four patients died, 3 of right-heart failure and 1 of sepsis. The acute hemodynamic 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 distance walked in 6 minutes improved by 148 m (95% CI, 4.5 to 282 m; P = 0.048). Of the 15 patients who continued to use inhaled iloprost, 8 stopped: Four 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 microg/d).
  
  
  CONCLUSIONS
  Inhaled iloprost may offer a new therapeutic option for improvement of hemodynamics and physical function in patients with life-threatening pulmonary hypertension and progressive right-heart failure that is refractory to conventional therapy.
authors.affiliation: Department of Internal Medicine II, Justus-Liebig-University, Giessen, Germany.
authors.forename: H
authors.initials: H
authors.lastname: Olschewski
authors.forename: H A
authors.initials: HA
authors.lastname: Ghofrani
authors.forename: T
authors.initials: T
authors.lastname: Schmehl
authors.forename: J
authors.initials: J
authors.lastname: Winkler
authors.forename: H
authors.initials: H
authors.lastname: Wilkens
authors.forename: M M
authors.initials: MM
authors.lastname: Höper
authors.forename: J
authors.initials: J
authors.lastname: Behr
authors.forename: F X
authors.initials: FX
authors.lastname: Kleber
authors.forename: W
authors.initials: W
authors.lastname: Seeger
mesh_terms: D000280:Administration, Inhalation; D000328:Adult; D000368:Aged; D017079:Exercise Tolerance / Q000187:drug effects; D005260:Female; D005500:Follow-Up Studies; D006333:Heart Failure / Q000188:drug therapy / Q000209:etiology; D006439:Hemodynamics / Q000187:drug effects; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D016285:Iloprost / Q000008:administration & dosage* / Q000009:adverse effects; D008297:Male; D008875:Middle Aged; D011659:Pulmonary Gas Exchange / Q000187:drug effects; D018709:Statistics, Nonparametric; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage* / Q000009:adverse effects
chemical_list: D014665:Vasodilator Agents; D016285:Iloprost