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Bosentan in pediatric patients with pulmonary arterial hypertension

Maurice Beghetti1

  • 1Hôpital des Enfants, Geneva, Switzerland. maurice.beghetti@hcuge.ch

Current Vascular Pharmacology
|April 10, 2009
PubMed

Insights

Bosutinib is an effective treatment for pediatric pulmonary arterial hypertension (PAH), improving long-term function and hemodynamics. While exercise capacity improvements are inconsistent, the drug is well-tolerated with fewer adverse events than in adults.

Area of Science:

  • Pediatric Cardiology
  • Pharmacology
  • Pulmonology

Background:

  • Pediatric pulmonary arterial hypertension (PAH) is a serious condition requiring effective treatment.
  • Bosentan is an endothelin receptor antagonist used in adult PAH, but its use in children requires evaluation.

Purpose of the Study:

  • To systematically review clinical studies on the safety and effectiveness of bosentan in pediatric PAH.
  • To provide an overview of current knowledge regarding bosentan therapy in children with PAH.

Main Methods:

  • A systematic literature search was conducted across MEDLINE, EMBASE, and BIOSIS from January 2000 to October 2007.
  • Keywords included 'pulmonary arterial hypertension', 'bosentan', and 'pediatric patients/children'.
  • 21 clinical studies (1 interventional, 11 observational, 9 case reports/series) were selected.

Main Results:

  • Bosentan demonstrated improvements in long-term functional status and hemodynamics in pediatric PAH patients.
  • Consistent improvement in exercise capacity was not observed.
  • Combination therapy with bosentan showed promising results, but guidelines are undefined.
  • Adverse events, including liver enzyme elevations, were less frequent than in adult studies.

Conclusions:

  • Uncontrolled studies suggest bosentan is a well-tolerated and effective treatment for pediatric PAH.
  • Further research, including controlled trials, is needed to establish definitive treatment guidelines.
Abstract

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