Approved Treatments for Pulmonary Hypertension
Although there is no cure yet for pulmonary hypertension (PH), there are a variety of therapies available that can help manage the symptoms of the disease and maintain or improve the quality of life of patients. Many of these therapies are aimed at relaxed and widening arteries to improve blood flow, resulting in lower blood pressure and increased oxygen flow around the body.
Adcirca (tadalafil) is used to ease symptoms of pulmonary arterial hypertension (PAH). It is designed to relax the smooth muscles of the arteries, leading to widening of the arteries and a reduction of blood pressure. This improves the transport of oxygen around the body, which can improve a patient’s ability to exercise.
Adempas (riociguat) was developed for chronic thromboembolic pulmonary hypertension (CTEPH) and PAH. It is designed to relax arterial muscle walls, which causes the arteries to widen, allowing blood to flow more freely. The result is lower blood pressure and increased oxygen flow around the body.
Flolan (epoprostenol GM) can be prescribed to PAH patients to improve exercise capacity and circulation, and ease symptoms. It is a synthetic variant of prostacyclin, a member of a group of molecules called prostaglandins, which naturally exist in the body and are involved in many biological functions, including dilating and relaxing blood vessels.
Letairis (ambrisentan) is approved to treat the symptoms of PAH both as a monotherapy and in combination with Adcirca in the U.S. and Europe. It is an endothelin type-A receptor antagonist that relaxes the muscles and helps widen the arteries, lowering blood pressure and allowing for oxygen to be transported more effectively around the body.
Opsumit (macitentan), an oral once-daily therapy, is approved to treat the symptoms and help slow the progression of PAH. It is also being investigated as a possible treatment for other types PH, including chronic thromboembolic pulmonary hypertension (CTEPH) and Eisenmenger syndrome. It is an endothelin receptor antagonist that allows muscles of the arterial walls to relax.
Orenitram is an extended-release oral formulation of treprostinil used to improve exercise ability and delay clinical worsening in PAH patients. It is a synthetic form of prostacyclin, which helps with the effective dilation of the arteries in the lungs and other parts of the body and inhibits blood cells called platelets from clumping and blocking arteries.
Remodulin (treprostinil) is a therapy used to treat PAH symptoms. It is administered as a continuous infusion or as an injection under the skin or into a vein. It is a man-made form of prostacyclin, which helps blood vessels relax and widen. Prostacyclin also prevents platelet activation and aggregation.
Revatio (sildenafil) is used to slow the progression of PAH and improve the ability to exercise. Revatio is a phosphodiesterase-5 (PDE-5) inhibitor that helps with vasodilation, leading to a reduction in blood pressure. This increases circulation through the lungs, which helps transport oxygen around the body and reduces the burden on the heart.
Tracleer (bosentan) is approved for the treatment of both adults and children with PAH. It is aimed at improving the ability of patients to exercise and slowing disease progression. It is an endothelin-1 inhibitor that helps relax and widen arteries, allowing for blood to flow more freely and oxygen to be transported more effectively around the body.
Tyvaso is an inhaled formulation of treprostinil administered via a hand-held, portable device. It is prescribed to ease PAH symptoms. In 2021, it became the first therapy approved in the U.S. for the treatment of PH associated with interstitial lung disease. Interstitial lung diseases are a large group of disorders characterized by fibrosis, or scarring, in the lungs.
Uptravi (selexipag) is intended to delay disease progression in patients with PAH. It mimics the effect of prostacyclin by binding and activating the receptors that normally bind prostacyclin. This binding causes blood vessels to dilate, decreasing blood pressure and improving pulmonary blood circulation.
Veletri (epoprostenol AS) is an intravenous treatment for PAH patients. It s synthetic version of prostacyclin, a hormone that PH patients’ bodies do not make enough of. It is designed to widen the blood vessels in the lungs, making oxygen-rich blood flow more easily.
Ventavis (iloprost) is an inhaled therapy used to improve the exercise capacity of people with PAH, potentially delaying or reducing worsening of the disease. It is a synthetic version of prostacyclin that is administered with a nebulizer. Because Ventavis is delivered directly to the lungs, it can do a better job of widening arteries and reducing blood pressure.