lung and airway disorders
Pulmonary Hypertension
Treatment of Pulmonary Hypertension
Treatment of the cause of pulmonary hypertension Often treatments to relieve symptoms, such as drugs that widen (dilate) blood vessels and supplemental oxygen Sometimes treatments to prevent or treat complications, such as anticoagulants and lung transplantation Treatment of pulmonary hypertension is best directed at the cause when the cause has been identified. Vasodilators (drugs to dilate blood vessels) work by reducing blood pressure in the pulmonary arteries. Vasodilators may improve quality of life, prolong survival, and prolong the time until lung transplantation needs to be considered. Before administering vasodilators, however, doctors may first test the effectiveness of these drugs while the person is in a cardiac catheterization laboratory because use of these drugs may be dangerous in some people. Vasodilators have not proved effective for people with pulmonary hypertension due to chronic obstructive pulmonary disease (COPD). In contrast, vasodilators are often helpful for pulmonary hypertension that occurs in people with the following: Idiopathic or inherited pulmonary hypertension Autoimmune disorders Chronic liver disease HIV infection Some congenital heart disorders Pulmonary hypertension caused by drugs or toxins Chronic thromboembolic pulmonary hypertension Vasodilators include drugs related to prostacyclin (which dilates the pulmonary artery), such asepoprostenolgiven intravenously,iloprostortreprostinilgiven via inhalation,treprostinilinjected under the skin, orselexipaggiven orally. Vasodilators can also be given by mouth, including phosphodiesterase-5 inhibitors (for example,sildenafilandtadalafil), endothelin-receptor antagonists (bosentan,ambrisentan, andmacitentan), and a guanylate cyclase stimulator (riociguat). Endothelin is a substance in the blood that causes constriction of the blood vessels. Phosphodiesterase inhibitors and the guanylate cyclase stimulator increase the ability of nitric oxide, a substance normally present in the body, to widen (dilate) the pulmonary artery. Sotatercept is a drug that helps treat common forms of inherited pulmonary hypertension. In people with pulmonary hypertension who have a low level of oxygen in the blood, the continuous use of oxygen through nasal prongs or an oxygen mask may reduce blood pressure in the pulmonary arteries and may relieve shortness of breath. A diuretic drug is usually given to assist the right ventricle in maintaining a normal volume for effective beating and to reduce leg swelling. An anticoagulant may also be given to reduce the risk of blood clots and subsequent Pulmonary Embolism (PE) . Lung and Heart-Lung Transplantation is an established procedure for treating people with pulmonary hypertension. Lung transplantation can be used only in people with severe disease who are healthy enough to withstand the potential consequences and difficulties with the procedure. 