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enrollment was too slow
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| Name | Class |
|---|---|
| Actelion | INDUSTRY |
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Pulmonary arterial hypertension (PAH), or high blood pressure in the lungs, is common in patients with congenital heart disease. Historically these patients suffered significant morbidity and mortality due to a lack of effective therapies. More recently, advanced therapies which target the mechanisms underlying the development and progression of PAH have been introduced into clinical care. Oral, intravenous, subcutaneous, and inhaled therapies are all available for the treatment of PAH. Patients with PAH are first treated with oral agents (including sildenafil and bosentan). However, if these agents fail to achieve the desired effect for the patient, intravenous or inhaled therapies may be initiated. Combination therapy with multiple agents is common in routine clinical care. However, the most efficacious therapeutic regimen has yet to be delineated. The present study seeks to evaluate the efficacy of one specific regimen: iloprost, an inhaled prostacyclin derivative, used in combination with oral therapy (sildenafil and/or bosentan). Iloprost has been approved by the FDA for use in this patient population. Adults with PAH already receiving oral therapy will be invited to participate in this study. Iloprost will be added to their current therapeutic regimen for a period of three months, with pre- and post-treatment assessments. These will include a cardiopulmonary exercise test, BNP (a blood test), six minute walking distance, and a quality of life questionnaire.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Iloprost | Experimental | Participants will be administered iloprost at 5 mcg/dose x 6 doses daily for 3 months. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Iloprost | Drug | Aerosolized iloprost, 5 mcg/dose x 6 doses daily for 3 months |
|
| Measure | Description | Time Frame |
|---|---|---|
| Safety and Tolerability | Number of Participants with adverse events, specifically mortality and heart failure. | 3 months |
| Measure | Description | Time Frame |
|---|---|---|
| Exercise Capacity | Change in exercise duration (modified Bruce protocol), maximal oxygen consumption (VO2 max), and/or VE/VCO2 ratio. | 3 months |
| Serum Brain Natriuretic Peptide (BNP) | Change in serum BNP level |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Jamil A Aboulhosn, MD | Ahmanson / UCLA Adult Congenital Heart Disease Center | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Ronald Reagan UCLA Medical Center | Los Angeles | California | 90095 | United States |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 19634081 | Background | Beghetti M, Tissot C. Pulmonary arterial hypertension in congenital heart diseases. Semin Respir Crit Care Med. 2009 Aug;30(4):421-8. doi: 10.1055/s-0029-1233311. Epub 2009 Jul 24. | |
| 15194174 | Background | Humbert M, Morrell NW, Archer SL, Stenmark KR, MacLean MR, Lang IM, Christman BW, Weir EK, Eickelberg O, Voelkel NF, Rabinovitch M. Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol. 2004 Jun 16;43(12 Suppl S):13S-24S. doi: 10.1016/j.jacc.2004.02.029. |
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| ID | Title | Description |
|---|---|---|
| FG000 | Iloprost | Participants will be administered iloprost at 5 mcg/dose x 6 doses daily for 3 months. Iloprost: Aerosolized iloprost, 5 mcg/dose x 6 doses daily for 3 months |
| Title | Milestones | Reasons Not Completed | ||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Overall Study |
|
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| ID | Title | Description |
|---|---|---|
| BG000 | Iloprost | Participants will be administered iloprost at 5 mcg/dose x 6 doses daily for 3 months. Iloprost: Aerosolized iloprost, 5 mcg/dose x 6 doses daily for 3 months |
| Units | Counts |
|---|---|
| Participants |
|
| Title | Description | Population Description | Parameter Type | Dispersion Type | Unit of Measure | Calculate Percentage | Denominator Units Selected | Denominators | Classes |
|---|---|---|---|---|---|---|---|---|---|
| Age, Categorical | Count of Participants |
| Type | Title | Description | Population Description | Reporting Status | Anticipated Posting Date | Parameter Type | Dispersion Type | Unit of Measure | Calculate Percentage | Time Frame | Units Analyzed | Denominator Units Selected | Arm/Group Information | Denominators | Classes | Analyses | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Primary | Safety and Tolerability | Number of Participants with adverse events, specifically mortality and heart failure. | Number of adverse events. | Posted | Number | participants | 3 months |
|
|
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| ID | Title | Description | Deaths (Affected) | Deaths (At Risk) | Serious Events (Affected) | Serious Events (At Risk) | Other Events (Affected) | Other Events (At Risk) |
|---|---|---|---|---|---|---|---|---|
| EG000 | Iloprost | Participants will be administered iloprost at 5 mcg/dose x 6 doses daily for 3 months. Iloprost: Aerosolized iloprost, 5 mcg/dose x 6 doses daily for 3 months |
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Enrollment was too slow. The trial was discontinued.
| Title | Organization | Phone | Extension | |
|---|---|---|---|---|
| Jamil Aboulhosn | UCLA | 310-825-5950 | jaboulhosn@mednet.ucla.edu |
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| ID | Term |
|---|---|
| D000081029 | Pulmonary Arterial Hypertension |
| D006330 | Heart Defects, Congenital |
| D004541 | Eisenmenger Complex |
| ID | Term |
|---|---|
| D006976 | Hypertension, Pulmonary |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D018376 | Cardiovascular Abnormalities |
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| ID | Term |
|---|---|
| D016285 | Iloprost |
| ID | Term |
|---|---|
| D011465 | Prostaglandins, Synthetic |
| D011453 | Prostaglandins |
| D015777 | Eicosanoids |
| D005231 | Fatty Acids, Unsaturated |
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| 3 months |
| Quality of Life | Change in quality of life as assessed by SF-36 QOL | 3 months |
| 10400015 | Background | Niwa K, Perloff JK, Kaplan S, Child JS, Miner PD. Eisenmenger syndrome in adults: ventricular septal defect, truncus arteriosus, univentricular heart. J Am Coll Cardiol. 1999 Jul;34(1):223-32. doi: 10.1016/s0735-1097(99)00153-9. |
| 9886728 | Background | Daliento L, Somerville J, Presbitero P, Menti L, Brach-Prever S, Rizzoli G, Stone S. Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J. 1998 Dec;19(12):1845-55. doi: 10.1053/euhj.1998.1046. |
| 9741570 | Background | Christman BW. Lipid mediator dysregulation in primary pulmonary hypertension. Chest. 1998 Sep;114(3 Suppl):205S-207S. doi: 10.1378/chest.114.3_supplement.205s. |
| 8532025 | Background | Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, Groves BM, Tapson VF, Bourge RC, Brundage BH, Koerner SK, Langleben D, Keller CA, Murali S, Uretsky BF, Clayton LM, Jobsis MM, Blackburn SD, Shortino D, Crow JW; Primary Pulmonary Hypertension Study Group. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med. 1996 Feb 1;334(5):296-301. doi: 10.1056/NEJM199602013340504. |
| 10733441 | Background | Badesch DB, Tapson VF, McGoon MD, Brundage BH, Rubin LJ, Wigley FM, Rich S, Barst RJ, Barrett PS, Kral KM, Jobsis MM, Loyd JE, Murali S, Frost A, Girgis R, Bourge RC, Ralph DD, Elliott CG, Hill NS, Langleben D, Schilz RJ, McLaughlin VV, Robbins IM, Groves BM, Shapiro S, Medsger TA Jr. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med. 2000 Mar 21;132(6):425-34. doi: 10.7326/0003-4819-132-6-200003210-00002. |
| 11897647 | Background | Simonneau G, Barst RJ, Galie N, Naeije R, Rich S, Bourge RC, Keogh A, Oudiz R, Frost A, Blackburn SD, Crow JW, Rubin LJ; Treprostinil Study Group. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med. 2002 Mar 15;165(6):800-4. doi: 10.1164/ajrccm.165.6.2106079. |
| 12151469 | Background | Olschewski H, Simonneau G, Galie N, Higenbottam T, Naeije R, Rubin LJ, Nikkho S, Speich R, Hoeper MM, Behr J, Winkler J, Sitbon O, Popov W, Ghofrani HA, Manes A, Kiely DG, Ewert R, Meyer A, Corris PA, Delcroix M, Gomez-Sanchez M, Siedentop H, Seeger W; Aerosolized Iloprost Randomized Study Group. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med. 2002 Aug 1;347(5):322-9. doi: 10.1056/NEJMoa020204. |
| 16946127 | Background | McLaughlin VV, Oudiz RJ, Frost A, Tapson VF, Murali S, Channick RN, Badesch DB, Barst RJ, Hsu HH, Rubin LJ. Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med. 2006 Dec 1;174(11):1257-63. doi: 10.1164/rccm.200603-358OC. Epub 2006 Aug 31. |
| 19475782 | Background | Krug S, Sablotzki A, Hammerschmidt S, Wirtz H, Seyfarth HJ. Inhaled iloprost for the control of pulmonary hypertension. Vasc Health Risk Manag. 2009;5(1):465-74. doi: 10.2147/vhrm.s3223. |
| 14556887 | Background | Hallioglu O, Dilber E, Celiker A. Comparison of acute hemodynamic effects of aerosolized and intravenous iloprost in secondary pulmonary hypertension in children with congenital heart disease. Am J Cardiol. 2003 Oct 15;92(8):1007-9. doi: 10.1016/s0002-9149(03)00991-3. |
| 19089967 | Background | Limsuwan A, Khosithseth A, Wanichkul S, Khowsathit P. Aerosolized iloprost for pulmonary vasoreactivity testing in children with long-standing pulmonary hypertension related to congenital heart disease. Catheter Cardiovasc Interv. 2009 Jan 1;73(1):98-104. doi: 10.1002/ccd.21793. |
| Participants |
|
| Sex: Female, Male | Count of Participants | Participants |
|
|
| Secondary | Exercise Capacity | Change in exercise duration (modified Bruce protocol), maximal oxygen consumption (VO2 max), and/or VE/VCO2 ratio. | unable to analyze because study was terminated prematurely and therefore follow up assessments were not obtained | Posted | 3 months |
|
|
| Secondary | Serum Brain Natriuretic Peptide (BNP) | Change in serum BNP level | unable to analyze because study was terminated prematurely and therefore follow up assessments were not obtained | Posted | 3 months |
|
|
| Secondary | Quality of Life | Change in quality of life as assessed by SF-36 QOL | unable to analyze because study was terminated prematurely and therefore follow up assessments were not obtained | Posted | 3 months |
|
|
| 0 |
| 3 |
| 0 |
| 3 |
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| D002318 | Cardiovascular Diseases |
| D006331 | Heart Diseases |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D005227 |
| Fatty Acids |
| D008055 | Lipids |
| D012898 | Autacoids |
| D018836 | Inflammation Mediators |
| D001685 | Biological Factors |