title: Efficacy and limitations of continuous intravenous epoprostenol therapy for idiopathic pulmonary arterial hypertension in Japanese children.
abstract: BACKGROUND
  There is little data on the long-term effects of continuous intravenous epoprostenol for children with idiopathic pulmonary arterial hypertension (IPAH) in Japan.
  
  
  RESULTS
  Thirty-one IPAH patients younger than 18 years old who had begun epoprostenol therapy at Toho University Omori Medical Center between January 1999 and June 2004 were reviewed. During a mean follow up of 3.4 years, the rate of those who survived or did not undergo a lung transplantation among the 27 patients who received home infusion therapy of epoprostenol was 100% at 1 year, 96.3% at 2 years, and 79.4% at 3 years. In 82% of survivors, the World Health Organization functional class was changed from III or IV to II according to improvements in the plasma brain natriuretic peptide level and the distance walked in 6 min during the follow-up period. In most cases, mean pulmonary artery pressure and the ratio of pulmonary to systemic vascular resistance remained high, although the cardiac index had improved to within a normal range 1 year after the initiation of epoprostenol. Therefore, sildenafil was administered as an additional therapy to 16 patients who presented with sustained severe PAH.
  
  
  CONCLUSIONS
  Continuous IV epoprostenol certainly improves survival and exercise tolerance in childhood IPAH, although the improvement of pulmonary vascular resistance regardless of long-term epoprostenol therapy is insufficient. Therefore, the addition of a new drug, such as sildenafil, is recommended to be administered in adjunction with epoprostenol.
authors.affiliation: The First Department of Pediatrics, Toho University School of Medicine, Tokyo, Japan.
authors.forename: Tomotaka
authors.initials: T
authors.lastname: Nakayama
authors.forename: Hiromitsu
authors.initials: H
authors.lastname: Shimada
authors.forename: Shinichi
authors.initials: S
authors.lastname: Takatsuki
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Hoshida
authors.forename: Takashi
authors.initials: T
authors.lastname: Ishikita
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Matsuura
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Saji
mesh_terms: D000293:Adolescent; D000959:Antihypertensive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D001794:Blood Pressure / Q000502:physiology; D002648:Child; D002675:Child, Preschool; D004305:Dose-Response Relationship, Drug; D011464:Epoprostenol / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000188:drug therapy* / Q000503:physiopathology; D007223:Infant; D007231:Infant, Newborn; D007262:Infusions, Intravenous; D007564:Japan; D020097:Natriuretic Peptide, Brain / Q000097:blood; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D020097:Natriuretic Peptide, Brain; D011464:Epoprostenol