Ignite Creation Date:
2024-05-05 @ 11:40 PM
Last Modification Date:
2024-10-26 @ 10:37 AM
Study NCT ID:
NCT01383083
Status:
UNKNOWN
Last Update Posted:
2011-06-28
First Post:
2010-11-29
Brief Title:
Effects of Iloprost Treatment in Adult Patients With Pulmonary Arterial Hypertension Related to Congenital Heart Disease
Sponsor:
Maryknoll Medical Center
Organization:
Maryknoll Medical Center
Study Overview
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
Status:
UNKNOWN
Status Verified Date:
2010-11
Last Known Status:
ACTIVE_NOT_RECRUITING
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
EIGER
Brief Summary:
The prevalence of PAH associated with congenital systemic-to-pulmonary shunts in Western countries has been estimated to range between 16 and 125 cases per million adults with 25-50 of this population affected by Eisenmengers syndrome The rarity of this syndrome combined with its complex pathophysiology account for the insufficient understanding of the principles underlying its proper treatmentRecent 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 Eisenmengers syndrome is based mainly on clinical experience rather than being evidence based Although Eisenmengers syndrome is uncurable disease the survival rate is relatively higher than primary PAH and the patients with Eisenmengers 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 Eisenmengers syndrome However there was scare data of outcomes of treatment with the inhaled iloprost in patients with Eisenmengers syndrome In Korea most of patients with Eisenmengers 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 Eisenmengers syndrome by the use of functional and hemodynamic parameters which would add the evidence of PAH-specific agents on the Eisenmengers syndrome
In this study we investigate to know the clinical benefit of iloprost on patients with Eisenmengers syndrome by the use of functional and hemodynamic parameters which would add the evidence of PAH-specific agents on the Eisenmengers syndrome
Detailed Description:
A large proportion of patients with congenital heart disease CHD in particular those with relevant systemic-to-pulmonary shunts will develop pulmonary arterial hypertension PAH if left untreated Persistent exposure of the pulmonary vasculature to increased blood flow as well as increased pressure may result in pulmonary obstructive arteriopathy which leads to increased pulmonary vascular resistance that if it approaches or exceeds systemic resistance will result in shunt reversal Eisenmengers syndrome the most advanced form of PAH associated with CHD is defined as CHD with an initial large systemic-to-pulmonary shunt that induces severe pulmonary vascular disease and PAH with resultant reversal of the shunt and central cyanosis The histopathological and pathobiological changes seen in patients with PAH associated with congenital systemic-to-pulmonary shunts such as endothelial dysfunction of the pulmonary vasculature are considered similar to those observed in idiopathic or other associated forms of PAH
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
None
Is a FDA Regulated Device?:
None
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None