I. What is pulmonary hypertension? Pulmonary hypertension is a group of pathophysiological syndromes characterized by elevated pulmonary artery pressure and pulmonary vascular resistance due to different etiologies. Common diseases causing pulmonary hypertension include idiopathic pulmonary hypertension, hereditary pulmonary hypertension, congenital heart disease, connective tissue disease, heart valve disease, chronic obstructive pulmonary disease, interstitial pulmonary fibrosis, chronic pulmonary embolism, metabolic diseases, vascular inflammatory diseases and fibrinous mediastinitis. Pulmonary hypertension is a large group of diseases involving multiple fields, multiple disciplines, and a wide range of people, and it has a high mortality and disability rate and a poor prognosis. According to the National Institutes of Health statistics of 194 patients with idiopathic pulmonary hypertension, the estimated mean survival was only 2.8 years. 1-year, 3-year and 5-year survival rates were 68%, 48% and 34%, respectively. Some studies suggest that the 3-year survival rate for patients with chronic thromboembolic pulmonary hypertension with a mean pulmonary artery pressure greater than 30 mmHg is only about 10%. Second, how to determine is pulmonary hypertension? Diagnosis basis: According to the 2009 American College of Cardiology Foundation/American Heart Association Expert Consensus on Pulmonary Hypertension and the 2009 European Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension. 1. Clinical manifestations: dyspnea, weakness, chest pain, syncope, edema. 2, auxiliary examination: electrocardiogram shows right-sided electrical axis, right ventricular hypertrophy; chest X-ray shows prominent pulmonary artery segment and widened right lower pulmonary artery; echocardiography indicates right atrial enlargement and increased pulmonary artery pressure; right heart catheterization confirms mean pulmonary artery pressure ≥ 25 mmHg. 3, common auxiliary tests for the diagnosis of pulmonary hypertension 1, laboratory tests: (1) blood routine; liver and kidney function, electrolytes; coagulation function, D-dimer; sedimentation (2) thyroid function; biochemical markers of heart failure (such as BNP or NT-Pro BNP); (3) blood gas analysis; rheumatological immunological indexes; (4) autoantibodies including anti-SSA, anti-SSB, anti-Sm, anti-Scl-70, anti-RNP, etc. (2) Chest X-ray and electrocardiogram: to clarify whether there is right ventricular hypertrophy, etc. 3.Cardiac ultrasound: to confirm the diagnosis of pulmonary hypertension associated with precordial disease and to exclude pulmonary hypertension caused by those with mitral stenosis. The degree of right heart dilatation, main pulmonary artery diameter, septal motion, and changes in ejection fraction of the right and left ventricles are observed to assess the condition and prognosis. 4.Right heart catheterization: a golden indicator for diagnosis, which can clarify pulmonary artery systolic pressure, diastolic pressure, mean pressure, small pulmonary artery wedge pressure, pulmonary vascular resistance, cardiac output, etc. 5.Pulmonary vascular CT 6.Pulmonary perfusion/ventilation imaging 7.6-minute walk test: to assess exercise tolerance. 4.How to treat pulmonary hypertension? According to the 2009 American College of Cardiology Foundation/American Heart Association Expert Consensus on Pulmonary Hypertension and the 2009 European Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension. 1. Management of right heart failure: (1) General management: oxygenation, monitoring of ECG, blood pressure and oxygen saturation of the fingertips. (2) Application of diuretics and digitalis preparations. (3) Application of vasoactive drugs: suitable for hemodynamic instability. 2.Anticoagulation therapy: suitable for partial arterial pulmonary hypertension and chronic pulmonary thromboembolic pulmonary hypertension. 3.Pharmacological treatment of pulmonary hypertension. (1) Calcium antagonists: Only patients with positive results of acute pulmonary vasodilation test can benefit from this treatment. (2) prostacyclin and its analogues (3) endothelin receptor antagonists (4) type 5 phosphodiesterase inhibitors V. Targeted therapy drugs for pulmonary hypertension 1. prostacyclin and its analogues: the treatment of pulmonary hypertension prostacyclin class of drugs are divided into nebulized inhalation, oral and intravenous three. Inhalation class – Wantavious (Iloprost), the drug can selectively act on the pulmonary vascular, half-life of 15 minutes, rapid onset of action, but the duration of action is short. The dose is at least 5-20ug per inhalation, 6 times a day. The oral class-beprostatin sodium, which is cheaper, obtains the effect of reducing pulmonary vascular pressure and resistance, improving exercise tolerance and increasing oxygen saturation by preventing intravascular thrombosis and diastolic pathways in the pulmonary vasculature. The intravenous application of iloprost or prostaglandin has the best therapeutic effect, but requires the application of intravenous cannula. 2, endothelin receptor antagonist: in China has been marketed endothelin receptor antagonist called bosentan (Bosentan, allcoli, Switzerland Acetelon company), the drug has the effect of dilating the pulmonary artery, reversing the formed pulmonary vascular injury and adjusting cardiac function. It is the only drug that has been shown in randomized, double-blind, placebo-controlled clinical studies to improve exercise tolerance, reduce pulmonary artery pressure and pulmonary vascular resistance in patients without causing significant side effects. The safety and efficacy of the drug are well assured in long-term application, except for the need for regular liver function testing. 3, type 5 phosphodiesterase inhibitors: sildenafil, vardenafil, tadalafil, etc., have the dual effect of dilating the pulmonary artery and improving cardiac function, and can improve the patient’s activity tolerance, improve oxygen saturation, reduce pulmonary artery pressure and small pulmonary artery resistance. Sildenafil 20mg/d treatment and vardenafil 5mg/d.