About 10-30% of precardiac disease can develop into pulmonary hypertension, characterized by progressive occlusion of the pulmonary vascular bed and heart failure, which severely limits surgical and interventional treatment. Pulmonary hypertension in combination with precordial disease is mainly characterized by shortness of breath after activity, palpitations and weakness, and if there is bruising of the mouth and lips after activity, it indicates that Eisenmenger syndrome has developed. If cyanosis and pestle-like fingers (toes) are present in left-to-right shunt type of precordial disease, it suggests Eisenmenger syndrome. The development of pulmonary hypertension in precardiac disease is divided into three stages: (1) left-to-right shunt stage (power pulmonary hypertension stage): the ratio of pulmonary circulation blood flow to body circulation blood flow (Qp/Qs) ≥ 1.5, pressure and resistance can be normal or elevated, but the total pulmonary resistance is mostly below 10 wood, which can be treated by surgery or intervention. (2) Bidirectional shunt stage: Qp/Qs between 1.0-1.5, pulmonary artery pressure and resistance are significantly elevated, total pulmonary resistance is above 10 wood, no longer suitable for surgery and intervention, but may still have the opportunity for surgery or intervention after drug treatment, drug treatment to reduce pulmonary artery pressure is the main. (3) Right-to-left shunt stage (Eisenmenger stage): Qp/Qs<1, total pulmonary resistance is significantly elevated, and the pulmonary vasculature shows irreversible lesions, which is currently an absolute contraindication to surgery, and there is no clear evidence whether drugs can delay life or even reverse its pressure. Right heart catheterization is the gold standard for confirming the diagnosis of pulmonary hypertension, providing a reliable basis for grading pulmonary hypertension and judging the effectiveness of treatment. It has been found that small pulmonary artery wedge pressure is significantly higher in the power type, suggesting left ventricular insufficiency, and lower in the resistance type. Acute pulmonary vasodilation test can assess prognosis and select therapeutic drugs. Patients with greater than 50% decrease in total pulmonary resistance have better prognosis than those with relatively low response, and calcium channel blockers are the ideal therapeutic drugs. The three drugs currently available for acute pulmonary vasodilator testing are epoprostenol, adenosine, and nitric oxide. In patients with high pulmonary artery resistance who already have a right-to-left shunt in combination with pulmonary hypertension, correction of the cardiac anomaly not only fails to reduce pulmonary artery pressure, but may cause a sharp rise in pulmonary artery pressure or even sudden death; some patients can sometimes be treated with interventional or surgical approaches to gradually reduce the left-to-right shunt, but clinical experience is currently insufficient. Pharmacological treatment is the only option for patients who have lost the opportunity for surgery and intervention. Conventional treatment includes oxygen, diuretics, digoxin and warfarin anticoagulation, etc. Pulmonary vasodilators include: (1) calcium channel blockers are used in patients with positive acute pulmonary vasodilation test results. (2) Angiotensin-converting enzyme inhibitors and angiotensin II receptor antagonists are most appropriate in the early stage of left-to-right shunt type, but not when pulmonary hypertension without heart failure or when resistance pulmonary hypertension develops. (3) Nitric oxide (NO) and its donor, postoperative inhalation of small doses of NO can significantly improve the patient's pulmonary hemodynamics and reduce the occurrence of pulmonary hypertension crisis, but not as a long-term therapeutic drug. (4) Phosphodiesterase inhibitors (milrinone, sildenafil, etc., vardenafil and tadanafil, etc.). (5) Prostaglandin analogues (epoprostenol and iloprost, etc.). (6) Endothelin receptor antagonists (bosentan and cetasentan, etc.). Other treatments include atrial septostomy and lung transplantation, which are suitable for patients with end-stage pulmonary hypertension.