For female patients, only non-pregnant women who are surgically sterile, postmenopausal or have documented infertility (over 50 years of age and amenorrheic for at least 1 year), or those of childbearing potential using one of the following methods of contraception:
* Barrier-type devices (e.g., condom, diaphragm) used ONLY in combination with a spermicide. A double-barrier method is recommended.
* Intrauterine devices (IUDs)
* Oral contraceptives, if used in combination with a barrier method
Body weight of 40 kg or higher
Patients diagnosed with connective tissue disease
Hemodynamics at rest, based on cardiac catheterization, should be as follows:
Hemodynamics during exercise, based on cardiac catheterization, should be as follows: mPAP \> 30 mmHg
Provide written informed consent
You may not be if
PAH associated with any other condition
Severe obstructive lung disease : FEV1∕ FVC \<0.5
Total lung capacity \<60% of normal predicted value
Unable or unwilling have a cardiac catheterization procedure
Acute or chronic impairment (other than dyspnea), limiting the ability to comply with study requirements (6-MWT)
Psychotic, addictive or other disorder limiting the ability to provide informed consent or to comply with study requirements
Moderate to severe hepatic impairment, i.e., Child-Pugh Class B or C
AST and ∕or ALT \> 3 times uln
Hemoglobin concentration \> 25% below the lower limit of normal
Systolic blood pressure \< 85 mm Hg
Pregnancy or breast-feeding
Treatment or planned treatment with another investigational drug
Treatment with an endothelin receptor antagonist, phosphodiesterase type 5 inhibitor, or with prostanoids (excluding acute administration during a catheterization procedure to test vascular reactivity) within 2 months of inclusion
Treatment with calcineurin-inhibitors (i.e., cyclosporine A and tacrolimus), fluconazole, glibenclamide (glyburide) within 1 week of study start;
Known hypersensitivity to bosentan or any of the excipients
Clareo Health | A Study to Evaluate the Use of Bosentan in Patients With Exercise Induced Pulmonary Arterial Hypertension Associated With Connective Tissue Disease
