Pulmonary hypertension (PH) is a severe disease diagnosed when the arteries responsible for transporting blood from the heart to the lungs become constricted. Under normal circumstances, blood is supposed to gather oxygen from the lungs and distribute it to the organs, muscles and body tissue. However, due to the disease, normal blood flow is disrupted and the heart becomes enlarged and weakened, which can lead to right heart failure. There are three types of pulmonary hypertension, which are pulmonary arterial hypertension (PAH), associated PH and idiopathic PH.
The diagnosis of different pulmonary hypertension types are related to the specific cause of the disease, which is important in offering the best treatment for patients. Despite the fact that there is currently no cure for pulmonary hypertension, there are drugs and treatments that can help decrease the progression of the disease. Knowing the underlying cause of PH helps physicians decide on the most appropriate therapy, but it is not always possible, which represents a major problem in treating the disease. When not treated, patients face a prognosis of only 2.8 years after diagnosis.
FDA-Approved Pulmonary Hypertension Medications
There are different types of treatments tailored to help PH patients manage their symptoms. Heart and lung transplants are the most extreme interventions in more severe cases of pulmonary hypertension. Breathing exercises, oxygen support, exercise therapeutic vests, lifestyle alterations and other treatments are also options. However, the use of medications are consistently used in almost every case of PH. There are different types of drugs designed to help PH patients avoid or delay heart failure and improve overall health.
Calcium-channel blockers help relax the blood vessels, while phosphodiesterase-5 inhibitors improve blood flow and decrease the burden of the overloaded heart. Digoxin helps strengthen the heart beat and diuretics are used to ease the problem of excess fluid accumulated in the feet and ankles due to the disease. There are currently seven medications available in the U.S. pharmaceutical markets that were approved by the U.S. Food and Drug Administration (FDA) between 2001 and 2013.
The latest drugs approved for the treatment of PH were Adempas (riociguat), which is commercialized by Bayer Healthcare Pharmaceuticals for PH, PAH and chronic thromboembolic, and Opsumit (macitentan), provided by Actelion Pharmaceuticals for PAH. Both of them were validated in the country in October 2013. Prior to that, Tyvaso (treprostinil) from United Therapeutics was approved in July 2009 and Adcirca (tadalafil) from Eli Lilly in May 2009.
In June 2007, Letairis (ambrisentan) from Gilead became available for PAH patients, five years after the approval of the Remodulin (treprostinil) drug from United Therapeutics, which is prescribed to PAH patients with NYHA Class II-IV symptoms. In addition, Tracleer (bosentan) has been on the markets since November 2001, commercialized by Actelion for patients with PAH.
Investigational Therapies For Pulmonary Hypertension
In addition to approved PH medications, there are also numerous pharmaceutical companies and investigators working on finding more effective treatments and a cure for pulmonary hypertension, including the Montreal Heart Institute, which is evaluating the drug PulmoBind for PH molecular imaging in a phase II study. Eli Lilly and Company and United Therapeutics are working with the University of Cincinnati and the University of North Carolina, Chapel Hill on Tadalafil for Sarcoidosis Associated Pulmonary Hypertension (SAPH).
Gilead Sciences is also in collaboration with the Medical University of South Carolina to study Ambrisentan (Letairis) for Sarcoidosis Associated Pulmonary Hypertension, while Pfizer is engaged in “A Study To Evaluate Safety And Efficacy Of IV Sildenafil In The Treatment Of Neonates With Persistent Pulmonary Hypertension Of The Newborn.” Other company working on a medication for PH is INO Therapeutics, which is evaluating the use of Nitric Oxide with the support of the National Institutes of Health Clinical Center (CC) of the National Heart, Lung, and Blood Institute (NHLBI).
In addition, the drug Milrinone is being studied in babies with persistent PH of the newborn by the University of Pennsylvania, Bedford Pharmaceuticals, the American Medical Association and the Thrasher Research Fund. M.D. Anderson Cancer Center is working on the use of Natrecor for patients with PH, and the Department of Veterans Affairs is investigating the drug Tadalafil for Pulmonary Hypertension Due to Chronic Lung Disease (TADA-PHILD).
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