Efficacy and Safety of Bosentan in Sickle Cell Disease (SCD) Patients With Pulmonary Arterial Hypertension (PAH)
Status:
Terminated
Trial end date:
2007-08-01
Target enrollment:
Participant gender:
Summary
The study will assess the effect of bosentan on pulmonary vascular resistance and exercise
capacity in sickle cell disease (SCD) patients diagnosed with pulmonary arterial
hypertension. It consists of 3 phases: Screening, Treatment and Follow-up. During the
Screening visit, the study doctor will decide if patients meet the study requirements. All
potential patients will have a diagnosis of increased pulmonary artery pressures that is
shown by right heart catheterization conducted shortly prior to start of study treatment.
Patients will be asked to perform exercise capacity test (walking as far as possible for 6
minutes). Following the Baseline visit, the treatment phase consists of 4 additional clinic
visits during which the good and bad effects of the drug are reviewed and exercise capacity
test will be repeated. Patients will be treated for 16 weeks. Blood samples will be collected
every month, or more often, if needed. At the end of the study, patients will be asked to
repeat the right heart catheterization and exercise capacity test. After completion of the
study, patients will have the option of enrolling in a long-term follow-up study where all
patients will receive active drug. Patients electing not to participate in the extension
study will be followed up for safety assessments for about 28 days after the end of the study
treatment.