6. Baseline PAH-therapy (Calcium channel blocker, bosentan, prostanoid, phosphodiesterase type-5 inhibitor) if present, has to be stable for at least 3 months prior to screening. During the study, all background treatments should remain stable
7. Signed informed consent by the parents or legal representatives
You may not be if
1. PAH etiologies other than listed above
2. Non-stable disease status
3. Need or plan to wean patient from intravenous epoprostenol or intravenous or inhaled iloprost
4. Systolic blood pressure \< 80% of the lower limit of normal range
5. Aspartate aminotransferase and/or alanine aminotransferase values \> 1.5 times the upper limit of normal range.
6. Moderate to severe hepatic impairment, i.e., Child-Pugh Class B or C
7. Hemoglobin and/or hematocrit levels \< 75% of the lower limit of normal range.
8. Known intolerance or hypersensitivity to bosentan or any of the excipients of the dispersible Tracleer tablet
9. Treatment with forbidden medication within 2 weeks or at least 5 times the half-life prior to randomization, whichever is the longest:
* Glibenclamide (glyburide)
* Cyclosporin A
* Sirolimus
* Tacrolimus
* Fluconazole
* Rifampicin (rifampin)
* Ritonavir
* Co-administration of CYP2C9 inhibitors (e.g., amiodarone, voriconazole) and moderate/strong CYP3A4 inhibitors (e.g., amprenavir, erythromycin, ketoconazole, diltiazem, itraconazole)
* Endothelin receptor antagonists (ERAs) other than bosentan
10. Treatment with another investigational drug within 1 month prior to randomization or planned treatment
Clareo Health | Effects of Two Dosing Regimens of Bosentan in Children With Pulmonary Arterial Hypertension
