This multicenter, open label, double-arm study aims at investigating the the continuous effect of long-Term rehabilitation training on clinical improvement(ITTCI) and cardiopulmonary function in pulmonary arterial hypertension patients

Pulmonary Arterial Hypertension (PAH) is a disease caused by various reasons leading to pulmonary vascular remodelling and then results in a progressive increase in pulmonary vascular resistance and right heart failure. PAH progresses rapidly,with a poor prognosis. Targeted drugs has brought about an improvement in the quality of life of patients with PAH. However, in most cases, patients' clinical symptoms will be gradually worsen with exercise capacity gradually declining. The prognosis of PAH remains bleak. Although exercise was previously considered as a relative contraindication for PAH patients , a growing body of research has found that exercise rehabilitation is safe and effective for them. Patients show greater improvement in their symptoms and quality of life. Effective and appropriate exercise rehabilitation in low- and middle-income countries can maximise the therapeutic benefits of targeted medication, improve patient survival rate, and enhance exercise tolerance and cardiorespiratory fitness. In this study, we will investigate the continuous effects of exercise rehabilitation on the clinical improvement (time to clinical improvement, TTCI) and cardiorespiratory fitness over a 1-year period of long-term rehabilitation, including aerobic training (aerobic power cycling and treadmill walking) and inspiratory muscle training (respiratory trainer and lip-contracting abdominal breathing).

Inclusion Criteria:

1. Patients who suffered from pre-capillary PAH (mPAP ≥ 20 mmHg, PVR > 2 Woods unit, PAWP ≤ 15 mmHg) confirmed by right heart catheterisation.
2. Patients who are in the status of WHO-FC I-III.
3. Patients between 18 and 75 years old
4. Female subjects are not pregnant.
5. Patients are treated with PAH-targeted drugs and are in a stable state and there is no progress of disease).
6. Patients have not received exercise rehabilitation training within six months.

Exclusion Criteria:

Patients with the following diseases or symptoms:

1. Pulmonary vascular occlusive disease
2. Respiratory diseases
3. Ischaemic heart disease, complex congenital heart disease (e.g., tetralogy of Fallot, etc.), cardiomyopathy, valvular disease
4. Active liver disease
5. Severe kidney disease
6. Motor disorders (e.g. lower limb fracture, ataxia, etc.)
7. Malignant tumour diseases
8. Physical disability
9. Hb ≤ 80g / L
10. Systolic blood pressure ≤85mmHg
11. History of syncope within 3 months
12. History of supraventricular or ventricular arrhythmia at rest within 3 months.

Because the specificity of rehabilization, it is not suitable for us to mask