title: Combination therapy of prostacyclin for pulmonary hypertension in congenital heart disease.
abstract: BACKGROUND
  Pulmonary arterial hypertension (PAH) is a recognized complication of congenital heart disease. Despite differences in etiology and pathophysiology, successful therapy for idiopathic PAH may benefit in patients with congenital heart disease. We theorized that combination of oral and aerosolization prostacyclin will benefit this group of patients in long-term.
  
  
  METHODS
  The study design was single group and open label study with intention to treat for patients with congenital heart disease with pulmonary artery (PA pressure) more than 50% of systemic pressure. All patients were given a combination of orally given beraprost sodium and inhalation of iloprost for 12 months. Data were collected prospectively consisting of functional class, O2 saturation, 6-minute walk test and right ventricular systolic pressure (RVSP).
  
  
  RESULTS
  There were 23 patients with an average right ventricular systolic pressure (+/- SD) of 94.8 +/- 14.5 mmHg and with average age of 27.8 +/- 14.9 years (2.5 to 50 years). The average oxygen saturation was 87.9 +/- 7 %. There were 12 patients with post surgical repair or cardiac catheterization interventional procedure and 11 with and Eisenmenger's syndrome. There were significant improvement of 6-minute-walk test from an average of 268 +/- 70 meters to 308 +/- 57 meters at the end of 12 months. The functional class of patients was also improving. However, there were no significant different in oxygen saturation.
  
  
  CONCLUSIONS
  Combination therapy of oral and inhalation of aerosolized vasodilators is a fascinating concept in the therapy of pulmonary hypertension. Treated patients showed an improvement in exercise capacity and right ventricular systolic pressure without a worsening in oxygen saturation.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand. sikdr@mahidol.ac.th
authors.forename: Kritvikrom
authors.initials: K
authors.lastname: Durongpisitkul
authors.forename: Decho
authors.initials: D
authors.lastname: Jakrapanichakul
authors.forename: Duangmanee
authors.initials: D
authors.lastname: Laohaprasitiporn
authors.forename: Jarupim
authors.initials: J
authors.lastname: Soongswang
authors.forename: Prakul
authors.initials: P
authors.lastname: Chanthong
authors.forename: Apichart
authors.initials: A
authors.lastname: Nana
mesh_terms: D000280:Administration, Inhalation; D000284:Administration, Oral; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D011464:Epoprostenol / Q000008:administration & dosage / Q000031:analogs & derivatives*; D005080:Exercise Test; D006330:Heart Defects, Congenital / Q000150:complications / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D016285:Iloprost / Q000008:administration & dosage*; D008875:Middle Aged; D010100:Oxygen / Q000097:blood; D014665:Vasodilator Agents / Q000008:administration & dosage*; D017725:Ventricular Pressure
chemical_list: D014665:Vasodilator Agents; C048081:beraprost; D011464:Epoprostenol; D016285:Iloprost; D010100:Oxygen