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Keep this study
Lead
Actelion
Age
18–85
Sex
ALL
Healthy volunteers
Not accepted
You may be eligible if
Signed informed consent prior to initiation of any study-mandated procedure.
Male or female patients aged 18-85 years.
Patients with symptomatic pulmonary arterial hypertension in New York Heart Association (NYHA) functional class III or IV at the time of initiation of iloprost inhalation (Ventavis®) therapy using the Power Disc-6 (PD-6).
Patients with the following types of pulmonary arterial hypertension (PAH) belonging to World Health Organization (WHO) Group I:
* 1.1: Idiopathic (IPAH)
* 1.2: Familial (FPAH)
* 1.3: Associated with (APAH)
* 1.3.1: Collagen vascular disease
* 1.3.2: Congenital systemic-to-pulmonary shunts at least 2 years post surgical repair
* 1.3.4: Human immunodeficiency virus (HIV) infection
* 1.3.5: Drugs and toxins
PAH confirmed by the most recent right heart catheterization showing:
* Mean pulmonary arterial pressure (mPAP)≥ 25 mmHg at rest
* Pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg or left ventricular end diastolic pressure (LVEDP) ≤ 15 mmHg. If both PCWP and LVEDP are available then the LVEDP value is retained for inclusion.
Compliant with a treatment regimen of commercial iloprost inhalation (Ventavis® 5 μg) using the I-neb® AAD® equipped with the PD-6 for at least 4 weeks prior to screening.
Pulmonary function tests (PFTs) including forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), and total lung capacity (TLC), performed within 6 months of screening.
If taking other medications for PAH, these must have been stable for 60 days prior to baseline.
If taking corticosteroids, these must have been stable for 60 days prior to baseline.
Women of childbearing potential with a negative urine pre-treatment pregnancy test at baseline and who:
* consistently and correctly use (from screening and up to 28 days after discontinuation of study drug) a reliable method of contraception with a Pearl index of \< 1%,
* are sexually abstinent, or
* have a vasectomized partner.
A woman is considered to have childbearing potential unless she meets at least one of the following criteria:
Previous bilateral salpingo-oophorectomy or hysterectomy
Premature ovarian failure confirmed by a specialist gynecologist
XY genotype, Turner syndrome, uterine agenesis
Is aged \> 50 years and not treated with any kind of hormone replacement therapy (HRT) for at least 2 years prior to screening, with amenorrhea for at least 24 consecutive months
Receipt of any prostacyclin or prostacyclin analog other than iloprost within 12 weeks before screening.
Anticipation of the need for intravenous prostacyclin use within 28 days of starting the Power Disc-15 (PD-15).
HIV-seropositive with any of the following:
* Concomitant active opportunistic infections within 6 months prior to screening
* Detectable viral load within 6 months of screening
* CD4+ T-cell count \< 200 mm\^3 within 3 months of screening
* Changes in antiretroviral regimen within 3 months of screening
* Anticipated changes in antiretroviral regimen during study periods 1 or 2
* Using inhaled pentamidine
Systemic hypotension with systolic blood pressure \< 95 mmHg.
Uncontrolled systemic hypertension (systolic blood pressure \> 160 mmHg or diastolic blood pressure \> 100 mmHg on repeated measurement).
History of left-sided heart disease, including any of the following:
* hemodynamically significant aortic or mitral valve disease
* restrictive or congestive cardiomyopathy
* left ventricular ejection fraction \< 40% by multigated radionucleotide angiogram (MUGA), angiography, or echocardiography
* coronary artery disease with continuing symptoms of angina pectoris
* life-threatening cardiac arrhythmias
Atrial septostomy within 1 year.
History of pulmonary embolism prior to diagnosis of PAH unless it can be documented that chronic thromboembolic pulmonary hypertension (CTEPH) has been specifically excluded (e.g., ventilation/perfusion (VQ) scan, pulmonary angiogram).
Restrictive lung disease: TLC \< 60% of normal predicted value.
Obstructive lung disease: forced expiratory volume/forced vital capacity (FEV1/FVC) \< 0.5 or clinically relevant chronic obstructive lung disease or asthma (including any patient requiring concomitant medication to control symptoms of bronchospasm including as needed (p.r.n.) use).
Clinically relevant bleeding disorder or active bleeding.
Moderate to severe hepatic impairment, i.e., Child-Pugh Class B or C or hepatic cirrhosis.
Pregnant or breast-feeding.
Chronic renal insufficiency, as defined by a creatinine of \> 2.5 mg/dL or the requirement for dialysis.
Hemoglobin \< 75% of the lower limit of normal range.
Any condition that prevents compliance with the protocol or adherence to therapy or ability to provide informed consent.
Participation in any other clinical trial, except observational, or receipt of an investigational product within 30 days prior to enrollment.
