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Doctors recommend pulmonary hypertension (PH) treatments based on several different factors, including what type of PH a person has, how much PH symptoms affect their daily life and limit their activities (referred to as “functional class”), age, other health conditions, and other medications they take. Treatment guidelines for PH generally specify that safer treatments should be tried first. If first-line treatments are not effective, doctors consider therapies with more serious side effects or a higher risk for complications.
Treatments for PH aim to reduce symptoms, preserve your ability to do daily activities, and slow the progression of disease. Medications, oxygen therapy, pulmonary rehabilitation, surgery, and lifestyle changes can all be used to treat PH.
Many classes of medication are used to manage PH. It’s common for people with PH to be prescribed drugs from multiple categories.
Prostaglandins are compounds made by the body that are similar to hormones. In cases of pulmonary arterial hypertension (PAH), a type of PH, lower levels of prostaglandins are associated with the narrowing of blood vessels in the lungs.
Health experts believe certain prostaglandins help treat PAH in two key ways. First, they dilate (widen) the blood vessels in the lungs, increasing blood flow and improving oxygen delivery to cells. Second, they have antiproliferative effects, which means they help prevent the excessive growth and thickening of cells in the blood vessel walls. This can reduce the narrowing of the blood vessels in the lungs, helping to maintain better blood flow and slow the progression of PAH.
Prostaglandins include:
Selexipag (Uptravi) is a prostacyclin receptor agonist. Prostacyclin is a type of prostaglandin. Scientists believe that prostacyclin receptor agonists work by mimicking the action of prostacyclin, rather than limiting its amount in the blood. By activating the prostacyclin receptor, these drugs help dilate blood vessels and inhibit the excessive growth of cells in the blood vessel walls, which can help manage pulmonary arterial hypertension.
Common side effects of prostaglandins include headache, nausea, flushed skin, and diarrhea. Rare but serious side effects include increased risk for bleeding.
Ambrisentan (Letairis), macitentan (Opsumit), and bosentan (Tracleer) are endothelin receptor antagonists (ERAs). Endothelin is a substance made by the body that causes the blood vessels to constrict.
Endothelin receptor antagonists primarily work by reducing the amount of endothelin in the blood. In people with PAH, ERAs can help slow disease progression and may even improve heart and lung function
Tadalafil (Adcirca, Tadliq, Alyq) and sildenafil (Revatio, Liqrev) are phosphodiesterase-5 (PDE5) inhibitors. Drugs in this class inhibit the action of an enzyme called “phosphodiesterase-5,” which works to control blood flow. Scientists believe PDE5 inhibitors help in treating PH by dilating the pulmonary arteries.
Macitentan and tadalafil (Opsynvi) is a combination of two drugs that helps reduce pressure in the pulmonary arteries.
Riociguat (Adempas) is a soluble guanylate cyclase (sGC) stimulator. It stimulates the action of the soluble guanylate cyclase enzyme, which works to dilate blood vessels. Soluble guanylate cyclase stimulators can improve exercise and function in adult cases of PAH and group 4 PH, also called “chronic thromboembolic PH (CTEPH).” In CTEPH, blood clots in pulmonary arteries cause high blood pressure in the lungs.
Commonly called “water pills,” diuretics are often one of the first drugs prescribed to treat people with hypertension (high blood pressure). Health experts believe diuretics work by helping get rid of excess fluid that puts pressure on the heart.
Common diuretics include furosemide (Lasix), bumetanide (Bumex), metolazone (Zaroxolyn), and spironolactone (Aldactone, CaroSpir). Diuretics are usually safe, but they can cause dehydration if you lose too much water.
Anticoagulants — known as “blood thinners” — can help prevent the formation of dangerous clots, which can cause a stroke or heart attack. Drugs from several classes can work in different ways to act as blood thinners.
A doctor may prescribe the blood thinner warfarin (Coumadin, Jantoven) to help prevent blood clots. Note that blood thinners can increase your risk of bleeding problems, and if you take them, you should undergo periodic blood tests to ensure the medication is working properly.
Scientists believe calcium channel blockers treat hypertension and chest pain by dilating the arteries and decreasing the force with which the heart pumps. However, these medications are typically effective only in a rare subset of people who respond well to vasodilators — medications that widen blood vessels.
Some examples of calcium channel blockers are:
Calcium channel blockers can cause side effects including constipation, headache, and palpitations (irregular heartbeat).
Digoxin (Digitek, Digox, Lanoxin) is a digitalis drug used to treat heart failure and atrial fibrillation. Health experts believe digitalis drugs work by increasing the force with which the heart beats and controlling irregular heart rhythms.
Sotatercept-csrk (Winrevair) is the first in a new class of drugs approved to treat adults with PAH, approved in March 2024 by the U.S. Food and Drug Administration (FDA). It works by improving the balance of blood vessel cell growth and proliferation (increase in number) of cells, which is a key factor in PAH. This drug, when added to existing therapy, has been shown to significantly improve exercise capacity and other important clinical outcomes for people with PAH.
In some cases, such as for individuals who are newly diagnosed with PH or PAH, doctors may recommend combination therapy. Combination therapy uses two or more medications to help treat PH symptoms. Monotherapy, in comparison, uses a single treatment or drug to treat PH. Combination therapy may include PDE5 inhibitors, sGC stimulators, ERAs, or prostacyclin and prostanoid medications.
Scientists are currently studying the safety and effectiveness of many new and existing drugs to treat PH and PAH. As of August 2024, treatments now or recently being studied for treating people with PH include:
Be on the lookout for some of these medications becoming available to people with PH in the coming years. If you’re interested in participating in a clinical trial for a new medication, speak to your pulmonologist.
People with group 3 PH — which is caused by conditions such as chronic obstructive pulmonary disease (COPD), interstitial lung disease, and sleep apnea — may develop hypoxia (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 available only by prescription.
Some people with hypoxia require oxygen therapy only when they are walking, eating, or sleeping or during airline flights. Others may have resting hypoxia and need supplemental oxygen even when they are sitting still.
When someone requires oxygen therapy 24 hours a day, it’s called “long-term oxygen therapy (LTOT).” People who receive 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:
People with PH may develop shortness of breath, which makes walking and performing other daily activities difficult. Pulmonary rehabilitation programs are designed to improve your ability to function and your general well-being. Pulmonary rehabilitation plans take into account a person’s individual condition, making the therapy appropriate for people with PH regardless of 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:
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, at a clinic, or in the home.
When PH is caused by cardiovascular disease or lung disease, surgery may help in treating the underlying problem. Other surgeries may improve symptoms in people with advanced PH who no longer respond to medications. In people with CTEPH, surgery can potentially cure their PH. Doctors recommend surgery based on the type of PH a person has, their age, and their overall health.
Some people with advanced pulmonary arterial hypertension 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 (providing short-term relief) while a person waits for a lung transplant.
Some cases of PH are caused by damage or a defect of the mitral valve, a small flap located between the two chambers on the left side of the heart that stops blood from flowing in the wrong direction.
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.
Pulmonary thromboendarterectomy (PTE) surgery is a potential cure for some people with CTEPH. 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.
Lung transplants may involve one lung (single-lung transplantation) or, more frequently, two lungs (bilateral lung transplantation) 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.
Wait time for a lung transplant is often two to three years. There are extensive criteria for becoming a candidate for organ transplant, including the severity of the person’s disease. Organ transplants can extend the lives of people who would otherwise die of PH. People who undergo organ transplants need to take immunosuppressants for the rest of their lives to prevent their bodies from rejecting the transplant.
Lifestyle changes can be an important part of managing PH symptoms.
Making healthy nutrition choices can be an important way to improve PH symptoms. Some changes your health care provider may recommend include:
Getting plenty of iron can also help make sure your blood can carry oxygen as effectively as possible. Talk with a health care provider before making any dietary changes to make sure they’re safe for you given your health status and medication regimen.
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, it’s suggested to 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 people with PH. Doctors recommend that individuals of childbearing age living with PH use an effective form of birth control. Birth control pills can raise a person’s risk for dangerous blood clots. You should discuss contraception options with your health care provider.
Getting regular vaccinations for flu and pneumonia can help prevent dangerous infections in those with PH.
Avoid situations and activities that can worsen PH symptoms, including:
On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 56,000 members come together to ask questions, give advice, and share their stories with others who understand life with pulmonary hypertension.
What treatments have you tried for PH? Which have been most effective? Share your experience in the comments below, or start a conversation by posting on your Activities page.
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I'm on a combination therapy of IV Remodulen, Winrevair, Letairus, Adcirca, and 2 diuretics. I was in a bad way but upping my dose of Remodulin and adding Winrevair has performed a miracle. A 6 minute… read more
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