Effects of Iloprost Treatment in Adult Patients With Pulmonary Arterial Hypertension Related to Congenital Heart Disease (EIGER)
|ClinicalTrials.gov Identifier: NCT01383083|
Recruitment Status : Unknown
Verified November 2010 by Maryknoll Medical Center.
Recruitment status was: Active, not recruiting
First Posted : June 28, 2011
Last Update Posted : June 28, 2011
The prevalence of PAH associated with congenital systemic-to-pulmonary shunts in Western countries has been estimated to range between 1.6 and 12.5 cases per million adults, with 25-50% of this population affected by Eisenmenger's syndrome. The rarity of this syndrome, combined with its complex pathophysiology, account for the insufficient understanding of the principles underlying its proper treatment.Recent decades have seen developments in pulmonary hypertension pathophysiology which have led to the introduction of new groups of drugs: prostacycline analogs (Epoprostenol, Treprostinil, Beraprost, Illoprost), phosphodiesterase inhibitors (Sildenafil, Tadalafil), endothelin receptor antagonists (Bosentan, Sitaxantan, Ambrisentan) and nitric oxide. These drugs should be administered to patients in III-IV NYHA class. Despite successful early results, the therapeutic effect on patients with Eisenmenger syndrome has not been conclusively established The treatment strategy for patients with PAH associated with congenital systemic-to-pulmonary shunts and, in particular, those with Eisenmenger's syndrome is based mainly on clinical experience rather than being evidence based. Although Eisenmenger's syndrome is uncurable disease, the survival rate is relatively higher than primary PAH, and the patients with Eisenmenger's syndrome are relatively younger group. So the improvement of exercise tolerance and quality of life is very important. Several randomized controlled trial reported favourable short- and long-term outcomes of treatment with the orally active dual endothelin receptor antagonist bosentan in patients with Eisenmenger's syndrome. However, there was scare data of outcomes of treatment with the inhaled iloprost in patients with Eisenmenger's syndrome. In Korea, most of patients with Eisenmenger's syndrome are treated with conservative therapy instead of administration of PAH-specific drug, because of lack of clinical experience. Moreover, oral agent such as bosentan, sidenafil is preferred than iloprost becase of more evidence and convenience. Our therapeutic efforts should be directed mainly towards preventing complications. As a rule, we should avoid agents with no established therapeutic efficacy and try to alleviate symptoms without any additional risk, so as not to disrupt the existing clinical balance.
In this study, we investigate to know the clinical benefit of iloprost on patients with Eisenmenger's syndrome by the use of functional and hemodynamic parameters, which would add the evidence of PAH-specific agents on the Eisenmenger's syndrome
|Condition or disease||Phase|
|Pulmonary Arterial Hypertension||Phase 4|
|Study Type :||Observational|
|Estimated Enrollment :||42 participants|
|Official Title:||Effects of Iloprost Treatment in Adult Patients With Pulmonary Arterial Hypertension Related to Congenital Heart Disease (Eisenmenger Physiology): Safety, Tolerability, Clinical, and Hemodynamic Effect.|
|Study Start Date :||November 2010|
|Estimated Primary Completion Date :||June 2011|
|Estimated Study Completion Date :||November 2012|
In the adult patient group, iloprost acceptable target dose is 2.5 ug 4-6 times/day according to the patient's compliance. Because of the concern of safety and tolerability, during the first 4 weeks of treatment, patients receive 2.5 ug twice daily. After 4 weeks, this is increased to the target dose, if iloprost is well tolerated.
- Pulmonary Vascular Resistance [ Time Frame: Change from Baseline in PVR at 24 weeks ]To identify hemodynamic benefit and safety of 24 weeks treatment with iloprost on patients with Eisenmenger's syndrom
- Assessment of quality of life [ Time Frame: Change from Baseline in QoL at 24 weeks ]To investigate the change of quality of life(SF-12) after 24 weeks treatment with iloprost
- Exercise capacity [ Time Frame: Change from baseline in exercise capacity at 24 weeks ]To investigate the change of exercise capacity(6-minute walking test) after 24 weeks treatment with iloprost
Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT01383083
|Principal Investigator:||Kyoung Im Cho, MD||Maryknoll General Hospital|