Iloprost for the Treatment of Pulmonary Hypertension in Adults With Congenital Heart Disease

NCT ID: NCT01319045

Last Updated: 2015-01-26

Basic Information

• Recruitment Status: TERMINATED
• Clinical Phase: NA
• Total Enrollment: 5 participants
• Study Classification: INTERVENTIONAL
• Study Start Date: 2011-06-30
• Study Completion Date: 2013-05-31

Brief Summary

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.

Conditions

Pulmonary Arterial Hypertension; Congenital Heart Disease; Eisenmenger's Syndrome

Study Design

• Allocation Method: NA
• Intervention Model: SINGLE_GROUP
• Primary Study Purpose: TREATMENT
• Blinding Strategy: NONE

Study Groups

Iloprost

Participants will be administered iloprost at 5 mcg/dose x 6 doses daily for 3 months.

Group Type: EXPERIMENTAL

Iloprost

Intervention Type: DRUG

Aerosolized iloprost, 5 mcg/dose x 6 doses daily for 3 months

Interventions

Iloprost

Aerosolized iloprost, 5 mcg/dose x 6 doses daily for 3 months

Intervention Type: DRUG

Other Intervention Names

Ventavis, prostacyclin analogue

Eligibility Criteria

Inclusion Criteria

• Age greater than or equal to 18 years old
• Congenital heart disease with pulmonary arterial hypertension and cyanosis (resting oxygen saturation < 90% on room air)
• Stable on oral therapy (PDE5 inhibitor and/or endothelin blockade) for at least three months

Exclusion Criteria

• Age < 18 years old
• Current intravenous or subcutaneous prostacyclin therapy
• Resting Systemic Hypotension (Systolic blood pressure < 85 mmHg)
• Women who are pregnant or may become pregnant (unwilling to utilize effective contraception), as well as nursing mothers
• Inability to ambulate
• Planned surgical procedure during the study period

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Actelion

INDUSTRY

Sponsor Role: collaborator

University of California, Los Angeles

OTHER

Sponsor Role: lead

Responsible Party

Jamil Aboulhosn

Assistant Professor of Medicine

Responsibility Role: PRINCIPAL_INVESTIGATOR

Principal Investigators

Jamil A Aboulhosn, MD

Role: PRINCIPAL_INVESTIGATOR

Ahmanson / UCLA Adult Congenital Heart Disease Center

Locations

Ronald Reagan UCLA Medical Center

Los Angeles, California, United States

Countries

United States

References

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.

Reference Type: BACKGROUND

PMID: 19634081 (View on PubMed)

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.

Reference Type: BACKGROUND

PMID: 15194174 (View on PubMed)

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.

Reference Type: BACKGROUND

PMID: 10400015 (View on PubMed)

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.

Reference Type: BACKGROUND

PMID: 9886728 (View on PubMed)

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.

Reference Type: BACKGROUND

PMID: 9741570 (View on PubMed)

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.

Reference Type: BACKGROUND

PMID: 8532025 (View on PubMed)

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.

Reference Type: BACKGROUND

PMID: 10733441 (View on PubMed)

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.

Reference Type: BACKGROUND

PMID: 11897647 (View on PubMed)

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.

Reference Type: BACKGROUND

PMID: 12151469 (View on PubMed)

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.

Reference Type: BACKGROUND

PMID: 16946127 (View on PubMed)

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.

Reference Type: BACKGROUND

PMID: 19475782 (View on PubMed)

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.

Reference Type: BACKGROUND

PMID: 14556887 (View on PubMed)

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.

Reference Type: BACKGROUND

PMID: 19089967 (View on PubMed)

Other Identifiers

Iloprost ACHD

Identifier Type: -

Identifier Source: org_study_id