Intravenous prostacyclin is a vasodilator . It helps patients who don't respond to treatment with calcium channel blockers given by mouth. It's continuously delivered by a portable, battery-operated infusion pump. Despite this complexity, prostacyclin improves pulmonary hypertension and permits increased physical activity, resulting in an improved quality of life for patients of all ages treated with prostacyclin from early infancy through the elderly. It's sometimes used as a bridge to help those patients waiting for a transplant, while in other cases it's used for long-term treatment.
Some patients also do well by taking medicines that make the work of the right ventricle easier.
Anticoagulants , for example, can decrease the tendency of the blood to clot, thus permitting the blood to flow more freely. Diuretics reduce the amount of fluid in the body, further reducing the amount of work the heart has to do. Some patients also require supplemental oxygen delivered through nasal prongs or a mask if breathing becomes difficult. Some need oxygen around the clock. The decision whether a patient requires heart-lung transplantation or lung transplantation is determined by a thorough evaluation at a lung transplantation center.