Effects of Iloprost Treatment in Adult Patients With Pulmonary Arterial Hypertension Related to Congenital Heart Disease

NCT01383083 | Unknown DMC

• 1 other identifier: EIGER2011
• Study Type: observational
• Target: 42
• Locations: 0 countries
• Sites: N/A

Brief Summary

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

Conditions

Pulmonary Arterial Hypertension

Keywords

Pulmonary arterial hypertension; Congenital heart disease; Eisenmenger physiology

Outcome Measures

Primary Outcomes (1)

• Pulmonary Vascular Resistance

  To identify hemodynamic benefit and safety of 24 weeks treatment with iloprost on patients with Eisenmenger's syndrom

  Change from Baseline in PVR at 24 weeks

Secondary Outcomes (2)

• Assessment of quality of life

  Change from Baseline in QoL at 24 weeks

• Exercise capacity

  Change from baseline in exercise capacity at 24 weeks

Study Arms (1)

iloprost

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.

Eligibility Criteria

• Age: 18 Years - 80 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

Patients with PAH associated with congenital systemic-to-pulmonary shuntand treated with iloprost for pulmonary hypertension : patients with PAH associated with corrected and uncorrected congenital systemic-to-pulmonary shunts and Eisenmenger's syndrome are included

You may qualify if:

• Patients with Eisenmenger syndrome are clinically stable and in World Health Organization functional class (WHO class) III or worse are selected for treatment.
• Eisenmenger syndrome is diagnosed echocardiographically as right-to-left shunting through the shunt defect.
• To exclude other causes of PAH, lung function tests (spirometry, forced expiratory volume in 1 second, and forced vital capacity)are obtained.

You may not qualify if:

• Patients with severe left ventricular dysfunction and/or pulmonary venous congestion (measured invasively or assessed echocardiographically) are excluded.
• Patients with obstruction of the right ventricular outflow tract, pulmonary valve, or pulmonary arteries or patients on prostacyclin, glibenclamide, or cyclosporine treatment were excluded.
• Patients with contraindication to Ventavis;
• Hypersensitive to Ventavis
• High risk of bleeding, which can be increased by use of Ventavis (e.g. active peptic ulcer, trauma, intracranial hemorrhage)
• Severe coronary disease, unstable angina, history of Acute myocardial infarction within 6 months, uncompensated heart failure not under close medical monitoring, severe arrhythmia, suspected pulmonary congestion, cerebrovascular disease within 3 months (e.g. transient ischemic attack, stoke)
• pulmonary hypertension due to venous occlusive disease
• valvular defect with dysfunction of cardiac muscle, which is independent of pulmonary hypertension
• pregnancy, women with high probability of pregnancy, breast feeding
• renal failure (creatinine clearance \<30mL/min)

Sponsors & Collaborators

• Maryknoll Medical Center: lead
• Pusan National University Hospital: collaborator
• Inje University: collaborator
• Yeungnam University Hospital: collaborator

MeSH Terms

Conditions

Pulmonary Arterial Hypertension; Heart Defects, Congenital

Condition Hierarchy (Ancestors)

Hypertension, Pulmonary; Lung Diseases; Respiratory Tract Diseases; Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• Kyoung Im Cho, MD

  Maryknoll General Hospital

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: CASE ONLY
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER

Study Record Dates

• First Submitted: November 29, 2010
• First Posted: June 28, 2011
• Study Start: November 1, 2010
• Primary Completion: June 1, 2011
• Study Completion: November 1, 2012
• Last Updated: June 28, 2011

Record last verified: 2010-11