Acute Haemodynamic Study of TPN171H in Patients with Pulmonary Arterial Hypertension

You may be eligible if

• Patients aged 18 to 75;
• Patients who have voluntarily decided to participate in this study, and signed the informed consent form;
• Patients who are able to understand and follow study plans and instructions;
• Patients with symtomatic PAH (Group1), a pulmonary vascular resistance (PVR) \> 3 Wood, a mean pulmonary artery pressure (mPVP) ≥25 mmHg and a pulmonary artery wedge pressure (PAWP) ≤ 15 mmHg either due to:
• 1. Idiopathic PAH (IPAH)
• 2. Familial PAH
• 3. Associated PAH due to drugs or toxins
• 4. Associated PAH due to connective tissue disease
• 5. Associated PAH due to congenital heart disease, if patients underwent surgical correction more than 1 year prior to screening
• Have a current diagnosis of being in WHO functional class II or III;
• Patients who are willing to take proper contraceptive during the study and within 3 months after the study completed.
• Moderate to severe COPD (FEV1 \< 60% predicted);
• Moderate to severe restrictive lung disease (FVC \< 70% predicted);
• Pre-treatment with PAH therapy within the last 1 months before the screening visit (including endothelin receptor antagonists, PDE5 inhibitors, guanylate cyclase agonist and prostacycline analogues);
• A "positive" response to acute vasodilator testing;
• Coagulation dysfunction (defined as activated partial thromboplastin time and international normalized ratio are both \>1.5 times upper limit normal) or patients with potential bleeding risk;
• Hepatic dysfunction indicated by: serum bilirubin\>3 times upper limit normal, ALT and AST\>2.5 times upper limit normal;
• Renal insufficiency (creatinine clearance\<30 mL/min)；
• Systolic blood pressure\<90 mmHg at screening;
• QT prolongation at screening, whose values (QTcF) exceeding 450 msec in males and 470 msec in females;
• Have enrolled in/or have a plan of an exercise training program for pulmonary rehabilitation before the screening visit/or during the study;
• Patients who have a recent (within 3 months) history of abusing alcohol or illicit drugs;
• Body weight\<40 kg；
• Patients who have participated in a clinical study involving another investigational drug within 1 month before the screening visit;
• For any other reasons that affect compliance with the study protocol, especially the long-term monitoring of floating catheters, according to the decision of investigators;
• HBV, HCV, HIV or Tp infection;
• Patients with gastrointestinal, urinary, reproductive, immunologic, endocrine, or central nervous system disease that, in the opinion of the investigator, will affect the study;
• Have a history of malignancies within 2 years, except for a cured basal cell or skin squamous cell carcinoma;
• Pregnant women, or breast feeding women;
• Patients with hypersensitivity to iloprost or any of the excipients.