The medical treatment of pulmonary arterial hypertension (PAH) has been difficult, and the prognosis for untreated PAH is poor, with an average survival time of approximately 2.8 years after diagnosis. It was not until 1992, when the first targeted drug, epoprostenol, was used in PAH, that the survival time and quality of life of patients have improved significantly. Currently, the main targeted drugs used in PAH are calcium antagonists, prostacyclin analogs, endothelin receptor antagonists, and phosphodiesterase inhibitors and newer drugs. Calcium antagonists Calcium antagonists are only profitable for patients with positive acute pulmonary vasodilator test, and have no effect on negative patients. The current application of calcium antagonists in China is very irregular, not only there is no basis for acute vasodilatation test, but also the drug selection is confusing. For positive patients calcium antagonists should be selected according to heart rate, with nifedipine for patients with slow basal heart rate and diltiazem for relatively fast patients. In terms of dose usage, application should be started at a small dose, increased to the maximum tolerated dose over a few weeks, and then maintained. Acute vasodilation tests should also be performed again after 1 to 2 years of application to re-evaluate sensitivity, and only those with long-term sensitivity should continue to apply. Prostacyclin analogs Since the introduction of prostacyclin for PAH, prostaglandin analogs such as travoprost, iloprost and beraprost have been used for PAH treatment. Studies have shown that these drugs can significantly improve patients’ symptoms and are well tolerated. Endothelin receptor antagonists Since 2001, when the non-selective endothelin receptor antagonist bosentan was used in the treatment of pulmonary arterial hypertension, the same type of drugs have been used in the treatment of PAH, including selective endothelin receptor antagonists such as sitaxsentan and ambrisentan, and currently only bosentan is available in China. Our registered indications for IPAH and scleroderma-related PAH, foreign indications for precardiac disease-related PAH and pediatric PAH, and many other studies have confirmed that bosentan can significantly improve exercise tolerance, WHO cardiac function classification, and hemodynamic indices in PAH patients, and is well tolerated. Phosphodiesterase inhibitors Phosphodiesterase (PDE) inhibitors can be classified into two major groups, relatively non-selective and selective, based on their characteristics of action on the PDE enzyme. Among them, sildenafil, a phosphodiesterase-5 inhibitor, is mainly used for erectile dysfunction treatment and was approved by the US FDA in 2005 for PAH pressure treatment. Studies and practice have confirmed that it can significantly improve patients’ cardiac function and is well tolerated. In addition to the above drugs for the treatment of pulmonary hypertension, some new drugs have emerged, including Rho kinase fasudil, guanylate cyclase Riociguat, sensitive K channel antagonist etacalcine, prostacyclin receptor agonist cicaprost, pentraxin carrier, vasoactive intestinal peptide (VIP), growth factor (VEGF, PDGF), etc. .