iloprost power disc-15 in pulmonary arterial hypertension

Conditions: Pulmonary Arterial Hypertension

Summary: a phase iiib, multicenter, open-label study of patients with pulmonary arterial hypertension treated with iloprost(inhalation)evaluating safety and inhalation times when converting from power disc-6 to power disc-15 with the i-neb® adaptive aerosol delivery® system (i-neb® aad®)


Eligibility criteria:
inclusion criteria: * 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. * pulmonary vascular resistance (pvr) \> 240 dyn-sec/cm\^5 * 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 exclusion criteria: * pah belonging to who group ii-v. * pah belonging to who group i other than that listed in the inclusion criteria, i.e., pah associated with: * 1.3.3: portal hypertension * 1.3.6: other (thyroid disorders, glycogen storage disease, gaucher disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative disorders, splenectomy) * 1.4: associated with significant venous or capillary involvement: * 1.4.1: pulmonary veno-occlusive disease (pvod) * 1.4.2: pulmonary capillary hemangiomatosis (pch). * 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.

Keywords: pulmonary arterial hypertension inhaled therapy power disc-15