A Study to Evaluate the Use of Bosentan in Patients With Exercise Induced Pulmonary Arterial Hypertension Associated With Connective Tissue Disease

Hamilton Health Sciences (HHS)

Status and phase

Unknown

Phase 3

Conditions

Hypertension, Pulmonary

Connective Tissue Disease

Treatments

Drug: bosentan

Study type

Interventional

Funder types

Other

Industry

Identifiers

NCT00864201

PAH-CTD-2007

Details and patient eligibility

About

The primary objectives of this exploratory study are to evaluate the effects of bosentan on hemodynamics (via cardiac catheterization) during exercise in patients with Pulmonary Arterial Hypertension (PAH) who have abnormal hemodynamics during exercise but normal hemodynamics at rest. The authors hypothesize that early treatment may change the course of disease progression by improving hemodynamics during exercise, thus delaying disease progression.

Enrollment

10 estimated patients

Sex

All

Ages

18+ years old

Volunteers

No Healthy Volunteers

Inclusion criteria

Men or women ≥ 18 years of age
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:
- Mean pulmonary arterial pressure (mPAP) : 18 - 25 mmHg
- PCWP ≤ 15 mmHg
Hemodynamics during exercise, based on cardiac catheterization, should be as follows: mPAP > 30 mmHg
Provide written informed consent

Exclusion criteria

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