Long-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt: does the beneficial effect persist?

Rosa Laura E van Loon, Elke S Hoendermis, Marielle G J Duffels, Anton Vonk-Noordegraaf, Barbara J M Mulder, Hans L Hillege, Rolf M F Berger

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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.

Original languageEnglish
Pages (from-to)776-82
Number of pages7
JournalAmerican Heart Journal
Issue number4
Publication statusPublished - Oct 2007


  • Adolescent
  • Adult
  • Antihypertensive Agents/administration & dosage
  • Child
  • Child, Preschool
  • Eisenmenger Complex/complications
  • Exercise Tolerance/drug effects
  • Female
  • Follow-Up Studies
  • Heart Defects, Congenital/complications
  • Humans
  • Hypertension, Pulmonary/drug therapy
  • Male
  • Middle Aged
  • Pulmonary Wedge Pressure/physiology
  • Sulfonamides/administration & dosage
  • Time Factors
  • Treatment Outcome
  • Vascular Resistance/physiology

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