Evaluation and Management of Pulmonary Hypertension in Noncardiac Surgery: A Scientific Statement From the American Heart Association | Circulation
Pulmonary Hypertension - University Of Utah Health
Pulmonary hypertension is a broad term because the condition can have many different causes. The World Health Organization classifies five types of pulmonary hypertension, based on the underlying cause:
Group 1—This is also known as pulmonary arterial hypertension (PAH). This type of pulmonary hypertension is rare. You may have inherited pulmonary hypertension. Or it may occur due to an autoimmune disease, an infection, or toxin exposure. One of the toxins that most commonly causes pulmonary hypertension is methamphetamine. A history of methamphetamine use increases your risk of pulmonary hypertension, even if you no longer use the substance. Some people also develop pulmonary hypertension for reasons that we do not understand (idiopathic pulmonary arterial hypertension).
Group 2—Heart disease affecting the left side of your heart, such as mitral valve disease, aortic valve disease, or heart failure causes this type. This is a more common cause of pulmonary hypertension.
Group 3—These types of pulmonary hypertension are caused by lung diseases such as chronic obstructive pulmonary disease (COPD) or interstitial lung disease. This type may also be caused by sleep apnea.
Group 4—You develop pulmonary hypertension because of chronic blood clots in the lungs.
Group 5—This group includes pulmonary hypertension caused by an assortment of conditions that don't have a well-understood link. This includes conditions such as blood disorders, metabolic disorders, sarcoidosis, and vasculitis.
Pulmonary Arterial Hypertension: Symptoms, Causes, And Treatments - WebMD
Pulmonary arterial hypertension (PAH) is a type of pulmonary hypertension. It means that you have high blood pressure in the arteries that go from your heart to your lungs. But it's different from having regular high blood pressure.
With PAH, the tiny arteries in your lungs become narrow or blocked. It's harder for blood to flow through them, and that raises the blood pressure in your lungs. Your heart has to work harder to pump blood through those arteries.
PAH is a progressive condition that gets worse as time goes on. After a while, the heart muscle gets weak. Eventually, it can lead to heart failure.
With pulmonary arterial hypertension, your heart works harder to get blood to your lungs, which can weaken your heart over time. (Photo Credit: iStock/Getty Images)
A few things can cause pulmonary arterial hypertension.
Genes may play a role in why some people get it. When that's the case, it's called heritable pulmonary arterial hypertension. Of people that have PAH, about 15%-20% have this heritable type.
Any of these can lead to pulmonary arterial hypertension:
Who else gets pulmonary arterial hypertension?It's most common in women who are between 30 and 60 years old. People of African and Hispanic descent are more likely to be affected.
You may not notice any symptoms for a while. The main one is shortness of breath when you're active. It usually starts slowly and gets worse as time goes on. You may notice that you can't do some of the things you used to without feeling tired and out of breath.
Other symptoms include:
If you have shortness of breath and see your doctor, they'll ask you about your medical history. They may also ask you:
Your doctor may order tests, including:
Your doctor may also do blood tests to check for HIV and conditions such as scleroderma or lupus.
If these tests show that you might have pulmonary hypertension, your doctor will need to do a right heart catheterization to be sure. Here's what happens during that test:
You doctor may also inject medicines into the catheter to see if the pulmonary arteries are stiff. This is called a vasoreactivity test.
Right heart catheterization is safe. Your doctor will give you a sedative and use local anesthesia. You can usually go home the same day, although you'll need someone to drive you home.
You may want to write down a list of questions before your appointment. That way, you can make sure you ask your doctor everything you want to. It can also help to have a friend or family member with you to help you take notes.
Some possible questions are:
Pulmonary arterial hypertension treatment differs from person to person, so your treatment plan will be specific to your needs. Ask your doctor what your options are and what to expect. PAH is rare, so your doctor may refer you to someone who specializes in pulmonary hypertension.
There are many types of medications for pulmonary arterial hypertension. Some you take by mouth, others you breathe in, and some you get through an IV. The goal of these medications is to balance the substances your lungs make. These include endothelin, nitric oxide, and prostacyclin. PAH medications can also help your lungs work better and make sure blood can move easier through your veins to you lungs.
The types of medications you may take for pulmonary arterial hypertension include:
Your doctor may also recommend other treatments for pulmonary arterial hypertension. These work alongside treatments such as medication to manage your symptoms. These include:
Pulmonary rehabilitation. This includes education about your condition so you can have the skills you need to manage your symptoms. You may learn how to safely be more active without feeling tired and out of breath. It can also boost your overall level of fitness. Pulmonary rehab is often a group setting, so you can meet other people who have PAH or similar conditions.
Other medications. Your doctor may suggest other kinds of medications, too. These include blood thinners to avoid clots and water pills (diuretics) to get rid of extra fluid. Oxygen therapy can help your body get the oxygen it needs.
Surgery. In some cases, your doctor may mention surgery, such as a lung or heart transplant. Other procedures, such as an atrial septostomy, can ease pressure on your heart.
One of the best things you can do for yourself is to stay active, even if you have shortness of breath. Regular exercise, such as taking a walk, will help you breathe better and live better. Talk to your doctor first to find out what kind of exercise is best for you and how much you should do. Some people may need to use oxygen when they exercise.
Get plenty of rest, too. Pulmonary arterial hypertension makes you tired, so get a good night's sleep, and take naps when you need to.
Just like anyone else, it's good for you to eat a nutritious diet with lots of fruits, vegetables, and whole grains. Limit how much salt you take, and try to avoid saturated fat and trans fat. That's important for your overall health.
Avoid things that put too much strain on your heart, including:
The Pulmonary Hypertension Association offers in-depth information on everything from medications to tips on making daily tasks easier. It also has an active online support community.
Pulmonary arterial hypertension is a condition that gets worse over time. But treatments can help your symptoms so you can live better. It may take some planning, but many people who have it find ways to do the things they love, just as they did before their diagnosis.
Remember that each person is different, and there are good treatments available. Work with your doctor to find what's right for you.
Can pulmonary arterial hypertension be reversed?No, pulmonary arterial hypertension can't be reversed. There's no cure for pulmonary arterial hypertension, but the earlier you get a diagnosis, the easier it is to live with.
What's the life expectancy of someone with pulmonary arterial hypertension?How long you can live with pulmonary arterial hypertension depends on many factors. On treatment, the average median life expectancy of someone with PAH is about six years after diagnosis.
What are some of the long-term complications of PAH?Some of the long-term problems of pulmonary hypertension, including PAH, are:
Pulmonary hypertension can also affect pregnancy. It can be dangerous for you and the fetus.
Pulmonary arterial hypertension is a type of pulmonary hypertension that gets worse over time. With PAH, you have high blood pressure in the arteries that go from your heart to your lungs. You may have symptoms such as shortness of breath, swelling, and chest pain, among other signs. Even though there's no cure, you can manage PAH symptoms with treatment, including medication and pulmonary rehab.
Here are some of the most commonly asked questions about pulmonary arterial hypertension.
How is PAH different from regular high blood pressure?Regular high blood pressure happens throughout your body, whereas pulmonary arterial hypertension is high blood pressure in your lungs. With regular high blood pressure (hypertension), your body's arteries narrow. With PAH, the arteries in your lungs narrow, which makes your heart work harder.
What causes idiopathic pulmonary arterial hypertension (IPAH)?Sometimes, doctors can't find a reason for high blood pressure in the lungs. In that case, the condition is called idiopathic pulmonary hypertension.
Is pulmonary arterial hypertension a disability?Yes, pulmonary hypertension, including PAH, is considered a disability. Experts recommend applying for disability benefits as soon as possible because the approval process can take months.
Winrevair For Pulmonary Arterial Hypertension: What You Need To Know
Winrevair, also known as sotatercept-csrk, is a medicine used to treat pulmonary arterial hypertension (PAH), which causes high blood pressure in the arteries that go from your heart to your lungs.
Winrevair is available as an injection given by a health care provider or by you or your caregiver after getting trained on the injection technique.
Below you'll find common questions and answers about Winrevair.
Pulmonary arterial hypertension is categorized into groups by the World Health Organization based on the cause of the symptoms. Winrevair is approved for the treatment of people who have Group 1 pulmonary arterial hypertension, which is caused by narrowness, thickening, or stiffness in the arteries in the lungs.
Pulmonary arterial hypertension is also categorized by functional class based on the types of physical activity people are able to perform. These are known as functional classes and they include I, II, III, and IV.
In people with pulmonary arterial hypertension, there is high blood pressure in the arteries that go from your heart to your lungs. This can cause your heart to work harder to pump blood through the arteries, which can cause your heart to become weaker over time. Over time, this may cause heart failure. Winrevair is the first medicine in a new class of medicines used in people with pulmonary arterial hypertension. It can open up narrowed blood vessels, which can help blood flow through more easily. As a result, you may be able to exercise more or perform ordinary activities with less symptoms.
Winrevair is an injection given subcutaneously, meaning just below the skin. It comes in a kit that contains the medicine in the form of a powder, a prefilled syringe that contains sterile water for injection, and supplies needed to mix and give the injection.
Your health care provider may give this medicine to you, or it can be given by yourself or a caregiver. Your health care provider will determine which option is best. If you or a caregiver will be giving the medicine, you will be trained on the proper way to mix and give the medicine. Follow all instructions that your health care provider gives you.
You will need to have blood taken for some lab tests before you receive your first five doses, and occasionally after that. Your health care provider will decide when you need to have your blood tested.
You will receive or give yourself this medicine every three weeks. The dose is based on your weight and may also be based on the results of laboratory tests. It can be given in the stomach (at least 2 inches from your belly button), the upper thigh, or the upper arm. Your health care provider will decide how often you will get treatment and what the dose will be.
Winrevair was studied in a trial that included over 300 people who had pulmonary arterial hypertension that was classified as Group 1, functional class II or III, by the World Health Organization. All people were receiving treatment for their pulmonary arterial hypertension for at least 90 days before starting the trial. They were allowed to continue this treatment during the trial. About half of the people in this trial received Winrevair and about half received a placebo injection, which did not contain any medicine.
The people who received Winrevair in this trial received 0.3 milligrams per kilogram of their weight for the first dose. Starting with the second dose, people received 0.7 milligrams per kilogram, which was continued unless the dose needed to be reduced.
The six-minute walk distance was used to determine the effects of Winrevair. This test measures how far you can walk in six minutes. This distance was measured for each person at the beginning of the trial and after 24 weeks to see if there was any improvement.
Here are some characteristics of people who participated in these studies.
People who were treated with Winrevair were able to walk about 34.4 more meters at the end of the 24-week trial compared to only 1 more meter for those who received the placebo injection.
The number of people who died or had a clinical worsening event was compared in this study. Clinical worsening event meant any symptom that led to patients becoming sicker, such as the need for a lung or heart transplant, the need for other medicines, the need for higher doses of medicine, hospitalization, surgery, or worsening of pulmonary arterial hypertension, for example.
The total number of people in this study who died or had at least one clinical worsening event was lower for people who received Winrevair than those who received the placebo injections. A total of nine people who received Winrevair died or experienced a clinical worsening event compared to 42 who received a placebo injection.
Two people died who were receiving Winrevair compared to seven who were receiving the placebo injections.
More people who received placebo injections had to add a new medicine or increase their dose compared to those who received Winrevair.
Placebo
Winrevair
10.6%
1.2%
More people who received a placebo had a hospital stay of at least a day for their pulmonary arterial hypertension compared to those who received Winrevair.
More people who received placebo had their pulmonary arterial hypertension get worse compared to those who received Winrevair.
Placebo
Winrevair
9.4%
2.5%
The most common side effects seen with Winrevair were headache, nosebleeds, rash, spider veins, diarrhea, dizziness, or skin redness.
To help prevent headaches, be sure to get enough sleep at night, exercise regularly, eat regular meals, drink plenty of water, and limit stress.
If you get a nosebleed, stay calm. Try not to lie down but lean a little forward to help keep the blood from draining down your throat. Pinch your nostrils to close them and hold for 5 to 10 minutes. You will need to breathe through your mouth. After the bleeding has stopped, gently blow your nose to remove any clots.
Compression stockings and physical exercise may help with spider veins. In addition, try not to stand or wear high heels for long periods of time. Elevate your legs when possible, and try not to cross them.
If you get diarrhea, you can try the BRAT diet – bananas, rice, applesauce and toast. Try to avoid foods that are spicy, fried, fatty, citrus, or vegetables that can cause gas (beans, cabbage, Brussels sprouts). Also avoid dairy and foods and drinks that are high in sugar or caffeine.
Contact your pharmacist or health care provider if you are experiencing dizziness. Be sure you are getting enough fluids and eating enough. Do not drive while you are feeling dizzy.
Winrevair may increase your hemoglobin levels or decrease your platelet levels. You may have to have your blood taken before your dose to measure your hemoglobin and platelets.
Winrevair may cause serious bleeding. Call your health care provider right away if you have any of the following signs of severe bleeding while taking Winrevair:
Do not take Winrevir if you are pregnant, as it can cause harm to a fetus. Talk to your health care provider if you are pregnant, planning to get pregnant, or become pregnant while using Winrevair. You should use effective birth control while taking Winrevair and for at least four months after your last dose.
Winrevair may affect your ability to get pregnant. Talk to your health care provider if this is an important concern for you.
These are not all of the possible side effects of Winrevair. Talk with your health care provider if you are having symptoms that bother you. If you experience anything that you think may be caused by Winrevair, you can also report side effects to the FDA at 800-FDA-1088 (800-332-1088).
Winrevair can be obtained from specialty pharmacies that will ship the medicine directly to you. The specialty pharmacy will work directly with you and your health care team. It is important to answer all calls that you receive from your specialty pharmacy.
There may be cost assistance to help you afford Winrevair. You may have options even if you do not have health insurance. The Merck Access Program provides insurance support during treatment and helps eligible people with financial assistance, if eligible. Your health care provider will help you get started. Call 888-637-2502 for more information.
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