Iloprost Power 15 in Pulmonary Arterial Hypertension

Inclusion Criteria:

- Signed informed consent prior to initiation of any study mandated procedure,

- Patients with symptomatic idiopathic, or familial pulmonary arterial hypertension or
pulmonary hypertension associated with human immunodeficiency virus (HIV) or
drugs/toxins in NYHA functional class II to IV.

- Women of childbearing potential must have a negative urine pregnancy test and must
use an adequate method of contraception during the study and for 28 days after
discontinuation of the study drug.

Exclusion Criteria:

- Pulmonary arterial hypertension related to any condition other than those specified
in the inclusion criteria,

- Pulmonary arterial hypertension associated with significant venous or capillary
involvement (Pulmonary capillary wedge pressure (PCWP) > 15 mmHg),

- Known pulmonary veno-occlusive disease, or pulmonary capillary hemangiomatosis,

- Moderate to severe obstructive lung disease: forced expiratory volume in 1
second/forced vital capacity (FEV1/FVC) < 70% and FEV1 < 65% of predicted value after
bronchodilator administration,

- Moderate to severe restrictive lung disease: total lung capacity (TLC) < 60% of
predicted value,

- Pregnant or breast-feeding women,

- Systemic hypertension (systolic blood pressure > 160 mmHg or diastolic blood pressure
> 100 mmHg on repeated measurement),

- Systolic blood pressure < 95 mmHg,

- Moderate to severe hepatic impairment, i.e., Child-Pugh Class B or C,

- Chronic renal insufficiency defined by serum creatinine > 2.5 mg/dL (221 μmol/L) or
ongoing dialysis,

- Clinically relevant bleeding disorder or active bleeding,

- For those patients on monotherapy, any contraindication to bosentan, ambrisentan, or
sildenafil, according to product label,

- Known hypersensitivity to iloprost or any of its excipients.