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inclusion/exclusion criteria limited enrollment
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| Name | Class |
|---|---|
| Pfizer | INDUSTRY |
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Pulmonary Arterial Hypertension (PAH) in the setting of Idiopathic Pulmonary Fibrosis(IPF)is a risk factor for morbidity and mortality in the peri-lung transplant(LT) setting. Currently there is no significant data to support the use of pulmonary vasodilators for PAH in the setting of interstitial lung disease such as IPF. The majority of IPF patients have PAH either at rest or during exercise. The study hypothesis is that sildenafil may improve morbidity and mortality in the peri-LT setting in both IPF cohorts with either resting or exercise PAH.
The purpose of this study is to evaluate the use of sildenafil in patients with pulmonary fibrosis and PH being considered for lung transplantation. We hypothesize that not only will sildenafil improve functionality and QOL in the pre-transplant setting but it may also improve primary graft dysfunction after lung transplantation.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Pre-transplant placebo | Placebo Comparator | There are two placebo comparators.... one for the group of patients with resting PAH and another for the group of patients with exercise PAH |
|
| Pre-transplant sildenafil | Experimental | There are two active comparators, one group with resting PAH and another with exercise PAH, both receiving drug. |
|
| Pre-transplant no PAH-specific therapy | No Intervention | this group of patients has no evidence for either resting or exercise PAH but will be followed without specific drug intervention |
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| sildenafil | Drug | the dose of sildenafil will be 20mg three times per day (orally) |
|
| Measure | Description | Time Frame |
|---|---|---|
| 6 minute walk distance (6MWD) change from Baseline | ATS guideline based 6MW distance | 6 months |
| Measure | Description | Time Frame |
|---|---|---|
| Right heart catheterization hemodynamics | pulmonary hemodynamics via invasive right heart catheterization | initial right heart catheterization compared to catheterization done on day of lung transplantation; we will specifically compare mean pulmonary artery pressure, pulmonary vascular resistance, and pulmonary artery wedge pressure |
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Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Rajan Saggar, MD | David Geffen School of Medicine, UCLA | Study Director |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| David Geffen School of Medicine UCLA | Los Angeles | California | 90095-1690 | United States |
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| Chemokine analysis on peripheral blood |
evaluation of a group of chemokines before and after the intervention in each arm |
| the chemokines will be quantified and compared between study entry and the time point just prior to lung transplantation |
| Quality of life assessment | SF-36 (short-form 36) | study entry compared to 6 months or at the time of lung transplantation (whichever comes first); there is a 0-100 scoring scale and the lower the score, the more disability |
| Quality of life assessment in the context of dyspnea | Saint George Respiratory Questionnaire (SGRQ) | the SGRQ will be be compared at two time points, study entry and at 6 months or the time of transplantation (whichever comes first); scores range from 0 to 100 and higher scores indicate more limitation |
| ID | Term |
|---|---|
| D000081029 | Pulmonary Arterial Hypertension |
| D054990 | Idiopathic Pulmonary Fibrosis |
| D017563 | Lung Diseases, Interstitial |
| D006976 | Hypertension, Pulmonary |
| ID | Term |
|---|---|
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D011658 | Pulmonary Fibrosis |
| D006973 | Hypertension |
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |
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| ID | Term |
|---|---|
| D000068677 | Sildenafil Citrate |
| ID | Term |
|---|---|
| D013449 | Sulfonamides |
| D000577 | Amides |
| D009930 | Organic Chemicals |
| D013450 | Sulfones |
| D013457 | Sulfur Compounds |
| D010879 | Piperazines |
| D006573 | Heterocyclic Compounds, 1-Ring |
| D006571 | Heterocyclic Compounds |
| D011687 | Purines |
| D006574 | Heterocyclic Compounds, 2-Ring |
| D000072471 | Heterocyclic Compounds, Fused-Ring |
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