Bosentan in Children With Pulmonary Arterial Hypertension Extension Study

An Open Label, Long-term, Safety, and Tolerability Extension Study Using the Pediatric Formulation of Bosentan in the Treatment of Children With Idiopathic or Familial Pulmonary Arterial Hypertension Who Completed FUTURE 1

The main objective of the FUTURE 2 study was to assess the long-term safety and tolerability of the pediatric formulation of bosentan in children with idiopathic pulmonary arterial hypertension or familial pulmonary arterial hypertension who completed FUTURE 1 study.

Inclusion Criteria:

• Signed informed consent by the parents or the legal representatives.
• Patients who completed the FUTURE 1 study.
• Patients who tolerated bosentan pediatric formulation and for whom bosentan is considered beneficial at the end of FUTURE 1.
• Males or females >= 2 and < 12 years of age at enrollment in FUTURE 2 (this study). Females who are menstruating must have a negative pregnancy test. A reliable method of contraception must be considered, if appropriate.

Exclusion Criteria:

• Intolerance to bosentan despite dose reductions.
• Any clinically significant laboratory abnormality that precludes continuation of bosentan therapy.
• Pregnancy or breast-feeding.
• Known hypersensitivity to bosentan or any of the excipients.
• Premature and permanent study drug discontinuation during FUTURE 1.