title: Long-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt: does the beneficial effect persist?
abstract: BACKGROUND
  Data on long-term response to bosentan in adults and especially children with pulmonary arterial hypertension (PAH) associated with systemic-to-pulmonary shunt are scarce.
  
  
  METHODS
  We studied bosentan efficacy in 30 patients (20 adults, 10 children) with the disease at short- (4 months), and long-term follow-up (through 2.7 years). World Health Organization functional class (WHO class), transcutaneous oxygen saturation, and 6-minute walk distance were assessed at baseline, 4 months, 1 year, 1.5 years, and at latest follow-up (median 2.7 years).
  
  
  RESULTS
  At baseline, children tended to have more severe disease compared with adults with regard to WHO class and congenital heart defects. At 4 months' follow-up, WHO class and 6-minute walk distance significantly improved in both adults and children. During long-term follow-up, this improvement persisted through 1 year but declined thereafter in the total group. In the children, a progressive decline in exercise capacity was observed from 1-year follow-up, whereas in the adults, improvement lasted longer. No change from baseline was seen in transcutaneous oxygen saturation. Three (10%) patients died, 2 (7%) discontinued bosentan, and 5 (17%) required additional PAH therapy (of whom 1 eventually died). One- and 2-year persistence of beneficial bosentan effect was 68% and 43% (total group), 78% and 57% (adults), and 50% and 20% (children), respectively.
  
  
  CONCLUSIONS
  Our experience with bosentan suggests short-term improvement in both adults and children with PAH associated with systemic-to-pulmonary shunt. At long-term follow-up, a progressive decline in beneficial bosentan effect was observed. The decline appeared most pronounced in the pediatric patients, who, in this study, tended to have more severe disease at baseline.
authors: Department of Pediatrics, Division of Pediatric Cardiology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
authors: Rosa Laura E
authors: RL
authors: van Loon
authors: Elke S
authors: ES
authors: Hoendermis
authors: Marielle G J
authors: MG
authors: Duffels
authors: Anton
authors: A
authors: Vonk-Noordegraaf
authors: Barbara J M
authors: BJ
authors: Mulder
authors: Hans L
authors: HL
authors: Hillege
authors: Rolf M F
authors: RM
authors: Berger
mesh_terms: D000293:Adolescent; D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D000077300:Bosentan; D002648:Child; D002675:Child, Preschool; D004541:Eisenmenger Complex / Q000150:complications; D017079:Exercise Tolerance / Q000187:drug effects; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000150:complications; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000401:mortality; D008297:Male; D008875:Middle Aged; D011669:Pulmonary Wedge Pressure / Q000502:physiology; D013449:Sulfonamides / Q000008:administration & dosage / Q000627:therapeutic use*; D013997:Time Factors; D016896:Treatment Outcome; D014655:Vascular Resistance / Q000502:physiology
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan