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Doctors recommend pulmonary hypertension (PH) treatments based on what type of PH you have, how much your PH symptoms limit your activities (referred to as functional class), your age, and any other health conditions or medications you take. Treatment guidelines generally specify that safer treatments should be tried first. If first-line treatments are not effective, doctors consider those with more serious side effects or a higher risk for complications.
Medications, oxygen therapy, pulmonary rehabilitation, surgery, and lifestyle changes can be used to treat PH.
Many classes of medication are used to manage PH. It is common for people with PH to be prescribed drugs from multiple categories. Read more details about each PH medication and its side effects in the Treatments A-Z pages.
Epoprostenol (sold under the brand names Flolan and Veletri), treprostinil (sold under the brand names Orenitram, Remodulin, and Tyvaso), and iloprost (Ventavis) are prostaglandins. Prostaglandins are hormone-like compounds made by the body. In cases of pulmonary arterial hypertension (PAH), a specific type of PH, lower levels of prostaglandins are associated with the narrowing of blood vessels in the lungs. In cases of PAH, prostaglandins are believed to work in two ways. First, prostaglandins dilate the blood vessels of the lungs, increasing the blood flow and the amount of oxygen the cells receive. Second, prostaglandins help slow scarring in the blood vessels of the lungs, delaying the progress of the disease.
Selexipag (Uptravi) is a prostacyclin receptor agonist. Uptravi is believed to work by mimicking the effect of prostaglandins.
Common side effects of prostaglandins include headache, nausea, flushed skin, and diarrhea. Rare but serious side effects of prostaglandins include increased risk for bleeding.
Ambrisentan (Letairis), macitentan (Opsumit), and bosentan (Tracleer) are endothelin receptor antagonists. Endothelin is a substance made by the body that causes the blood vessels to constrict. Endothelin receptor antagonists are believed to work by limiting the amount of endothelin in the blood. In cases of PAH, endothelin receptor antagonists may slow disease progression and potentially reverse some heart and lung damage caused by the disease.
Tadalafil (Adcirca) and sildenafil (sold under the brand names Revatio and Viagra) are phosphodiesterase-5 (PDE-5) inhibitors. Drugs in this class inhibit the action of the enzyme phosphodiesterase-5, which works to control blood flow. In cases of PH, PDE-5 inhibitors are believed to work by dilating the pulmonary arteries.
Riociguat (Adempas) is a soluble guanylate cyclase stimulator (SGCS). Adempas stimulates the action of an enzyme called soluble guanylate cyclase, which works to dilate blood vessels. Adempas can improve exercise and function in adult cases of PAH and group 4 (chronic thromboembolic PH or CTEPH).
Commonly called water pills, diuretics are often one of the first drugs prescribed to treat people with hypertension (high blood pressure). Diuretics are believed to reduce blood volume by ridding your body of sodium and water through urination. Common diuretics include furosemide (Lasix), Metolazone, and spironolactone (Aldactone). Diuretics are usually safe, but they can cause dehydration if you lose too much water.
Anticoagulants — known as blood thinners — can help prevent dangerous clots from forming and causing stroke or myocardial infarction (heart attack). Drugs from several classes can work in different ways act as blood thinners. One of the most common blood thinners is the familiar aspirin, a nonsteroidal anti-inflammatory product sold over the counter. Other blood thinners include clopidogrel (Plavix), warfarin (Coumadin), and aspirin and extended-release dipyridamole (Aggrenox). Blood thinners can raise the risk of bleeding problems.
Calcium channel blockers are believed to treat hypertension and chest pain by dilating the arteries and decreasing the force with which the heart pumps. Some examples of calcium channel blockers are amlodipine (Norvasc), diltiazem (sold under the brand names Cardizem and Tiazac), and verapamil (sold under the brand names Calan, Verelan, and Covera-HS). Calcium channel blockers can cause side effects including constipation, headache, and palpitations.
Digoxin, a digitalis drug, is used to treat heart failure and atrial fibrillation. Digitalis drugs are believed to work by increasing the force with which the heart beats and controlling irregular heart rhythms. Digoxin is sold under the brand names Lanoxin and Digitek.
People with group 3 PH caused by conditions such as chronic obstructive pulmonary disease (COPD), interstitial lung disease, and sleep apnea may develop hypoxia, or low levels of oxygen in the blood. If you are diagnosed with hypoxia, you may benefit from supplemental oxygen through oxygen therapy. Oxygen therapy is only available by prescription.
Some people with hypoxia only require oxygen therapy when they are walking, eating, sleeping, or during airline flights. Others may have resting hypoxia and need supplemental oxygen even when they are sitting still. When someone needs oxygen therapy 24 hours a day, this is known as long-term oxygen therapy (LTOT). Those who require LTOT must use it at least 15 hours a day to experience benefits. However, for maximum benefits, those who need LTOT should use oxygen therapy 24 hours a day.
There are three main types of oxygen delivery devices: oxygen-gas cylinders, oxygen concentrators, and liquid-oxygen devices. Read more about oxygen therapy.
People with PH may develop shortness of breath that makes it difficult to walk and perform other daily activities. Pulmonary rehabilitation is a program designed to improve your ability to function and general well-being. Because pulmonary rehabilitation plans take into account your individual condition, pulmonary rehabilitation is appropriate for people whose PH is at any functional class.
Pulmonary rehabilitation programs focus on exercises that improve strength, lung function, and ability to walk and perform daily activities. They may include components of education such as nutrition counseling, breathing and energy conservation strategies, and guidance on how to manage your PH. Some pulmonary rehabilitation programs also offer psychological support. Additionally, by attending a pulmonary rehabilitation program, you can meet others with lung disease who are learning how to improve their condition.
Pulmonary rehabilitation may take place at a hospital, clinic, or in the home. Read more about pulmonary rehabilitation.
When PH is caused by cardiovascular disease or lung disease, surgery may be performed to treat the underlying problem. Other surgeries may improve symptoms in people with advanced PH that no longer responds to medications. In people with group 4 PH, surgery can potentially cure PH. Doctors recommend surgery based on the type of PH and your age and overall health. Read more details about each surgery in the Treatments A-Z pages.
Some people with advanced pulmonary arterial hypertension (PAH) and severe right heart failure may be candidates for atrial septostomy surgery. Atrial septostomy can relieve pressure in the right side of the heart and reduce PAH symptoms. Atrial septostomy may be done as a palliative measure while a person with PAH waits for a lung transplant.
The goal of atrial septostomy surgery is to create a small hole between the right and left atria of the heart to decompress the overburdened right side of the heart. Atrial septostomy is commonly done under conscious sedation. Atrial septostomy is usually performed in a cardiac catheterization laboratory, a specialized examination room in a hospital for heart-related tests and procedures. After atrial septostomy surgery, you will need to spend about 48 hours being observed in the hospital’s intensive care unit (ICU). You will likely receive supplemental oxygen.
In some people with PH, the disease is caused by damage or a defect of the mitral valve of the heart. Mitral valve surgery can reduce PH symptoms in certain cases. Mitral valve surgery may be performed alone or during another heart surgery such as coronary artery bypass grafting. The mitral valve may be repaired or replaced. Valves may be replaced with either artificial (also known as mechanical or prosthetic) valves or biological valves made of human or animal tissue. Read more about mitral valve surgery.
Pulmonary thromboendarterectomy (PTE) surgery is a potential cure for some people with group 4 PH (also known as chronic thromboembolic PH or CTEPH). CTEPH is a type of PH wherein blood clots in pulmonary arteries cause high blood pressure in the lungs. During PTE surgery, surgeons remove the blood clots from the pulmonary arteries.
A lung transplant may be an option for some people with PH whose condition is progressing and for whom medical therapy is no longer effective. A transplant for PH rarely involves the heart and lungs.
Lung transplants may involve one (single-lung transplantation) or more frequently two (bilateral lung transplantation) lungs harvested from an organ donor who has died recently. Less commonly, two living donors can each donate one lobe of their lungs for transplantation into a smaller person — usually a child — with PH.
In any type of transplantation, the donor must be a good genetic match to avoid organ rejection. The organs must be harvested from an organ donor who has died recently. The donor must also be a similar size and located within your geographical region. Wait time for a lung transplant is often two to three years. There are extensive criteria for becoming a candidate for organ transplant in addition to the severity of your disease.
Organ transplant extends the life of people who would otherwise die of PH. You will need to take immunosuppressants for the rest of your life to prevent your body from rejecting the heart transplant.
For people with PH, making good nutrition choices can be an important way to improve symptoms. Limiting salt intake, carefully monitoring fluid intake, and maintaining a healthy weight can make a significant difference in how you feel. Getting plenty of iron can help make sure your blood can carry oxygen as effectively as possible.
Years ago, doctors believed that people with PH should avoid exercise, as they thought too strenuous a regimen could lead to heart problems. More recently, most PH specialists have come to believe that regular sessions of gentle exercise are safe and likely beneficial for those with PH. Even small amounts of physical activity may help improve symptoms such as fatigue, shortness of breath, dizziness, faintness, swelling, and irregular heart rhythm.
If you smoke, quit. Smoking raises blood pressure, damages red blood cells and blood vessels, and makes blood more likely to clot, which can cause strokes and heart attacks.
Pregnancy can be life-threatening for women with PH. Women of childbearing age are recommended to use an effective form of birth control. However, birth control pills can raise the risk for dangerous blood clots. Women with PH should discuss contraception options with their doctors.
Getting regular vaccinations for flu and pneumonia can help prevent dangerous infections in those with PH.
Avoid situations that can worsen PH symptoms, including:
Kelly Crumrin is a senior editor at MyHealthTeam and leads the creation of content that educates and empowers people with chronic illnesses. Learn more about her here.
