Ambrisentan for the Improvement in Right Ventricular Strain in Scleroderma Associated Pulmonary Arterial Hypertension

• Status: Terminated
• Trial end date: 2017-05-25
• Target enrollment: 0
• Participant gender: All

Summary

This research study is looking at the use of the drug ambrisentan and if it can improve right ventricle function in people with systemic sclerosis-associated pre-pulmonary arterial hypertension. It is also looking at using right ventricle function changes as a marker of disease severity.

• Phase: N/A
• Accepts Healthy Volunteers?: No

Details

• Lead Sponsor: National Jewish Health
• Treatments: Ambrisentan

Criteria

Inclusion Criteria:

• Age >18 years, < 80 years

• Systemic sclerosis with any of the following features:

1. Duration of Raynaud's phenomena >8 years

2. Anticentromere antibody positivity

3. isolated nucleolar-pattern ANA positivity

4. Extensive telangiectasias

5. DLCO < 60% in the absence of extensive ILD

6. FVC%/DLCO% >1.6

7. Unexplained dyspnea

• Right heart catheterization-proven pre PAH (mean PAP 20-25 mmHg and pulmonary capillary wedge pressure <15 mmHg)

• Systolic blood pressure >100 mmHg

• Reliable contraception for women of childbearing age

• Informed consent

Exclusion Criteria:

• < 18 years or > 80 years

• Left ventricular ejection fraction < 55%

• Systolic or diastolic left ventricular congestive heart failure

• Liver disease (abnormal AST/ALT, chronic hepatitis, or cirrhosis)

• Extensive ILD or FVC< 60%

• Pregnant

• Breast-feeding women

• Cyclosporine use