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.

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

Primary (idiopathic and familial) pulmonary hypertension and PAH secondary to scleroderma without significant interstitial pulmonary disease classified as New York Heart Association (NYHA) functional class III and IV