Bosentan in Children With Pulmonary Arterial Hypertension Extension Study

This study has been completed.
Information provided by (Responsible Party):
Actelion Identifier:
First received: April 26, 2006
Last updated: April 8, 2013
Last verified: April 2013
This is a multicenter, multinational, open label, non-comparative, phase III extension study 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.

Condition Intervention Phase
Pulmonary Hypertension
Drug: bosentan
Phase 3

Study Type: Interventional
Study Design: Allocation: Non-Randomized
Endpoint Classification: Safety/Efficacy Study
Intervention Model: Single Group Assignment
Masking: Open Label
Primary Purpose: Treatment
Official Title: 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 AC-052-365 (Future 1)

Resource links provided by NLM:

Further study details as provided by Actelion:

Primary Outcome Measures:
  • Treatment-emergent adverse events [ Time Frame: up to 24 hours after discontinuation ] [ Designated as safety issue: Yes ]
  • Adverse events leading to premature discontinuation of study drug [ Time Frame: discontinuation of study ] [ Designated as safety issue: Yes ]
  • Serious adverse events [ Time Frame: up to 28 days after permanent discontinuation of study drug ] [ Designated as safety issue: Yes ]
  • Changes from Baseline in vital signs, body weight, and height [ Time Frame: End of Study ] [ Designated as safety issue: Yes ]
  • Treatment-emergent marked laboratory abnormalities [ Time Frame: Discontinuation of study ] [ Designated as safety issue: Yes ]

Secondary Outcome Measures:
  • Change from Baseline from FUTURE 1 of WHO functional class, quality of life questionnaire, Global Clinical Impression Scale according to parents/legal guardians and physician [ Time Frame: Study End or premature study drug discontinuation ] [ Designated as safety issue: No ]

Enrollment: 33
Study Start Date: August 2005
Study Completion Date: March 2012
Primary Completion Date: October 2011 (Final data collection date for primary outcome measure)
Arms Assigned Interventions
Experimental: 1
32 mg breakable tablets
Drug: bosentan
32 mg breakable tablets 4 mg maintenance dose
Other Name: Tracleer


Ages Eligible for Study:   2 Years to 12 Years
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No

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.
  Contacts and Locations
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Please refer to this study by its identifier: NCT00319020

United States, Colorado
The Children's Hospital Cardiac Care Center
Denver, Colorado, United States, 80218
United States, New York
Columbia University Medical Center
New York, New York, United States, 10032
Hopital Antoine Beclere
Clamart, France, 92140
Hopital Necker
Paris, France, 75743
CHE de Toulouse Hopital d'Enfants
Toulouse, France
Deutsches Herzzentrum
Augustenburger, Germany
Universitats Kinderklinik
Giessen, Germany
Policlinico S. Orsola-Malpighi
Bologna, Italy, 40138
Beatrix Children's Hospital
Groningen, Netherlands
Hopital des Enfants
Geneva, Switzerland
United Kingdom
The Institute of Child Health
London, United Kingdom
Sponsors and Collaborators
  More Information

Responsible Party: Actelion Identifier: NCT00319020     History of Changes
Other Study ID Numbers: AC-052-367  FUTURE 2 
Study First Received: April 26, 2006
Last Updated: April 8, 2013
Health Authority: United States: Food and Drug Administration

Keywords provided by Actelion:
pulmonary arterial hypertension

Additional relevant MeSH terms:
Hypertension, Pulmonary
Cardiovascular Diseases
Lung Diseases
Respiratory Tract Diseases
Vascular Diseases
Antihypertensive Agents
Cardiovascular Agents
Pharmacologic Actions
Therapeutic Uses processed this record on April 27, 2016