How long can one live with severe pulmonary hypertension is related to whether the primary disease can be effectively controlled, and if timely treatment can significantly extend the survival period. Severe pulmonary hypertension refers to the resting state of the pulmonary artery pressure is greater than 45mmHg. If it is caused by congenital heart disease, at this time, it is considered to have reached the late stage of the disease, which may be accompanied by serious cardiac insufficiency, and may even be cardiac arrest, sudden death, which seriously affects the patient’s survival time. Active drug treatment, such as ipratropium and iloprost, can help reduce pulmonary hypertension, thereby improving the patient’s condition and prolonging survival. Lung transplantation or heart-lung transplantation can also be considered if internal medicine treatment is not effective. Patients with severe pulmonary arterial hypertension should not exert themselves while undergoing active treatment, ensure adequate sleep, and refrain from traveling to high-altitude areas. When discomfort occurs, prompt medical attention is needed to avoid delays.