IPAH HPAH和药物引起的PAH患者急救员to acute vasoreactivity tests and with WHO FC I/II and sustained haemodynamic improvement (same or better than achieved in the acute test) after at least 1 year on CCBs only |
Long-term-treated historical PAH patients with monotherapy (>5–10 years) stable with low-risk profile |
IPAH patients >75 years old with multiple risk factors for heart failure with preserved LVEF (high blood pressure, diabetes mellitus, coronary artery disease, atrial fibrillation, obesity) |
PAH patients with suspicion or high probability of pulmonary veno-occlusive disease or pulmonary capillary haemangiomatosis |
Patients with PAH associated with HIV infection or portal hypertension or uncorrected congenital heart disease, as they were not included in RCTs of initial combination therapy |
PAH patients with very mild disease (e.g.WHO FC I, PVR 3–4 WU, mPAP <30 mmHg, normal right ventricle at echocardiography) |
Combination therapy unavailable or contraindicated (e.g.severe liver disease) |