Inhaled Iloprost in the Treatment of Patients With Pulmonary Hypertension up to 4 Years

Study Overview
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Brief Summary
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This is an open-label, uncontrolled, prospective long-term observation of Specific Drug in the treatment of patients with pulmonary hypertension up to 4 years. 160 patients with primary (idiopathic and familial) pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) due to scleroderma with New York Heart Association (NYHA) functional class and exercise capacity, defined as class III and IV planned to enroll. Efficacy, safety and tolerability of the drug and the survival of the patients will be observed.

Official Title
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Open-label, Uncontrolled, Prospective Long-term Observation of Inhaled Iloprost in the Treatment of Patients With Pulmonary Hypertension up to 4 Years

Conditions
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Pulmonary Hypertension

Intervention / Treatment
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* Drug: Ventavis inhaled (Iloprost, BAYQ6256)


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients who has pulmonary hypertension treated with inhaled iloprost older than 18 years old. Primary (idiopathic and familial) pulmonary hypertension (PH) with New York Heart Association (NYHA) functional class and exercise capacity, defined as class III and IV. Pulmonary arterial hypertension (PAH) due to scleroderma with New York Heart Association (NYHA) functional class and exercise capacity, defined as class III and IV. Patients who signed written informed consent. Exclusion Criteria: Severe coronary heart disease or unstable angina; Myocardial infarction within the last six months; Decompensated cardiac failure if not under close medical supervision; Severe arrhythmias; Cerebrovascular events (e.g. transient ischaemic attack, stroke) within the last 3 months. Pulmonary hypertension due to venous occlusive disease. Congenital or acquired valvular defects with clinically relevant myocardial function disorders not related to pulmonary hypertension. Pregnancy and lactation Age below 18

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Group 1<br> | Drug: Ventavis inhaled (Iloprost, BAYQ6256)<br>* Since this study is an observational, inhaled iloprost dosage and frequency for each patient will be prescribed by physicians as medically required.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| 6 Minutes walking distance (change in meters) |  | At month 48 | 
| Adverse events, weight, vital findings |  | At month 48 | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| 6 Minutes walking distance (% change) |  | At month 48 | 
| New York Heart Association Functional Class |  | At month 48 | 
| Change in drug dosage or frequency, need for PAH specific drug combination, need for drug change |  | At month 48 | 
| Pulmonary hypertension related hospitalization |  | At month 48 | 
| Heart and/or lung transplantation, mortality |  | At month 48 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Pulmonary hypertension, Inhaled iloprost, Efficacy, Safety, Tolerability, Survival, NYHA class III and IV