The human circulatory system is divided into the body circulation and the pulmonary circulation. Increased blood pressure in the body circulation is often referred to as hypertension, while higher than normal pressure in the pulmonary circulation is called pulmonary hypertension. The diagnosis of idiopathic pulmonary hypertension consists of two aspects: determining the increased pulmonary artery pressure and screening for the cause of the increased pulmonary artery pressure. The clinical manifestations of pulmonary hypertension are usually nonspecific, with early weakness and progression to dyspnea, chest pain, hemoptysis, palpitations, syncope, swelling and hoarseness. Since the causes of pulmonary hypertension are quite numerous, the clinical manifestations will be corresponding. Therefore, careful and comprehensive history and physical examination are important to obtain the correct diagnosis. Clinically, electrocardiogram and chest radiograph can suggest the diagnosis of pulmonary hypertension. The most valuable screening method is cardiac ultrasound, but cardiac ultrasound is an estimation method and is influenced by many factors in determining pulmonary hypertension. The definitive method for the diagnosis of pulmonary hypertension is right heart catheterization, which is the most important test for pulmonary hypertension and provides many important hemodynamic indices in addition to accurate measurement of pulmonary artery pressure, as well as determining the patient’s susceptibility to treatment with calcium antagonists. The diagnosis of idiopathic pulmonary hypertension requires the exclusion of other causes, such as: hereditary, drug-related, connective tissue diseases (systemic lupus erythematosus, mixed connective tissue disease, systemic sclerosis, and rheumatoid arthritis), congenital heart disease, various heart diseases, lung diseases, and thromboembolic pulmonary hypertension. Therefore, patients need to undergo many tests, such as CT pulmonary angiography or pulmonary perfusion ventilation scan to determine the presence of pulmonary embolism. For the treatment of idiopathic pulmonary hypertension, general basic therapy is very important, with oxygen therapy, diuretics, digoxin and anticoagulants depending on the condition. Calcium antagonists such as nifedipine and diltiazem may be used if cardiac catheterization is indicated. Drugs directly targeting pulmonary hypertension are currently available in the Chinese market and can be used in one or a combination of the following depending on the condition. (1) Prostacyclin: Iloprost (Vantave) 10-20 mg, inhaled, 6-9 times daily. (2) Endothelin receptor antagonists: bosentan (allcolytic) 125 mg orally twice daily and 62.5 mg orally twice daily for the first 4 weeks. Use of bosentan requires monitoring of liver function once a month. With Anrisentan (vanrelix), adverse liver function reactions are less frequent. (3) Type 5 phosphodiesterase inhibitors: Sildenafil 20-25mg orally, 3 times daily. Vardenafil 5mg orally twice daily. Studies have demonstrated that the choice of drugs that directly target pulmonary hypertension can effectively improve the prognosis of idiopathic pulmonary hypertension. Due to the high cost of medications for pulmonary hypertension, there is currently a charity drug supply program for Vantanavir and Bosentan. If there are other causes of pulmonary hypertension, aggressive management of co-morbidities is also required.