Exercise Induced Pulmonary Hypertension in Systemic Sclerosis and Treatment With Ambrisentan: A Prospective Single Center, Open Label, Pilot Study
| City: |
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Los Angeles |
| State: |
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California |
| Zip Code: |
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90095 |
| Conditions: |
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Systemic Sclerosis - Shortness of Breath - Pulmonary Hypertension |
| Purpose: |
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The purpose of this study is to determine the clinical characteristics and hemodynamic
profiles that predict exercise induced pulmonary hypertension in 15 patients with systemic
sclerosis. The study also aims to determine the effectiveness of Ambrisentan for subjects
with exercise induced Pulmonary Arterial Hypertension (PAH) with scleroderma
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| Study Summary: |
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The current literature addresses therapies for patients with resting PAH only, diagnosed by
right heart catheterization. However, the World Health Organization (WHO) also recognizes
and defines exercise induced pulmonary arterial hypertension (ex-PAH), which may precede the
development of resting PAH. The natural progression of PAH, especially during exercise, has
not been well delineated. An exercise hemodynamic study previously showed that in normal
healthy subjects the mean pulmonary pressure does not exceed 30mmHg even at maximal cardiac
outputs. A prior study evaluated exercise Doppler echocardiography systemic sclerosis
patients with normal resting echocardiograms, finding an abnormal response which was defined
as an estimated right ventricular systolic pressure greater than 40 mmHg. In the same study,
6.6% of the patients progressed to resting PAH over the followup period of 12 months.
Limited data is available regarding the prevalence of ex-PAH in systemic sclerosis using
right heart catheterization.
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| Criteria: |
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Inclusion Criteria:
1. Systemic Sclerosis diagnosed by the American College of Rheumatology consensus
statement including any of the following:
- Limited
- Diffuse
- Sine Scleroderma
2. Patients must be willing and able to undergo right heart catheterization with lower
extremity cycle ergometry
3. Mean pulmonary artery pressure (mPAP) > 30mmHg with exercise; PCWP ≤ 15mmHg on RHC at
rest
4. Men and women, ages 18 years of age or older
5. Standard adjunctive medications will be allowed concurrently in this study at the
discretion of the treating pulmonologist and rheumatologist, including digoxin,
diuretics, anticoagulants (e.g. warfarin), stable immunosuppression or other
anti-fibrotic therapy for at least one month prior to enrollment
Exclusion Criteria:
1. Resting PAH (mPAP > 25mmHg) on right heart catheterization
2. Other known causes of PAH including prior venous thromboembolism, HIV infection,
chronic liver disease with portal hypertension, left ventricular systolic dysfunction
(e.g. LVEF < 40%), and congenital causes of PAH
3. Severe hepatic disease precluding the use of ambrisentan (AST/ALT ≥3x ULN).
4. Women who are pregnant or breastfeeding.
5. Concurrent therapy with a prostanoid or prostanoid analogue, PDE5 inhibitors, or
enrolled in another active clinical study.
6. Use of any prostacyclin or endothelial receptor antagonist (ERA) within 30 days
before study entry.
7. Bed or wheel chair bound or a baseline 6-Minute Walk distance (6MWD) less than 150
meters.
8. Childbearing capable women who are unwilling or unable to use an acceptable method to
avoid pregnancy for the entire study period.
9. New York Heart Association (NYHA) Classification: Class IV
10. Renal dysfunction (serum creatinine >2.5mg/dL).
11. Uncontrolled sleep apnea.
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| NCT ID: |
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NCT01051960 |
| Primary Contact: |
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Principal Investigator
Rajeev Saggar, MD
University of California, Los Angeles
Rajeev Saggar, MD
Phone: 310-825-5635
Email: rasaggar@mednet.ucla.edu
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| Backup Contact: |
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Email: ABechtel@mednet.ucla.edu
Amber Betchel
Phone: 310-825-0425
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| Location Contact: |
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Los Angeles, California 90095
United States
Amber Betchel
Phone: 310-825-0425
Email: abetchel@mednet.ucla.edu
Site Status: Recruiting |
| Data Source: |
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ClinicalTrials.gov |
| Date Processed: |
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May 19, 2013 |
| Modifications to this listing: |
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