A Simple 6-Minute Walk Test is Used for Determining The Severity of Pulmonary Arterial Hypertension
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Exercise testing is used to discover the severity of Pulmonary Hypertension. This testing consists of either a 6-minute walk test or a cardiopulmonary exercise test.
A 6-minute walk test measures the distance you can quickly walk in 6 minutes. A cardiopulmonary exercise test measures how well your lungs and heart work while you exercise on a treadmill or bicycle.
During exercise testing, your doctor will rate your activity level. Your level is linked to the severity of your PH. The rating system ranges from class 1 to class 4.
- Class 1 has no limits. You can do regular physical activities, such as walking or climbing stairs. These activities don't cause PH symptoms, such as tiredness, shortness of breath, or chest pain.
- Class 2 has slight or mild limits. You're comfortable while resting, but regular physical activity causes PH symptoms.
- Class 3 has marked or noticeable limits. You're comfortable while resting. However, walking even one or two blocks or climbing one flight of stairs can cause PH symptoms.
- Class 4 has severe limits. You're not able to do any physical activity without discomfort. You also may have PH symptoms while at rest.
Over time, you may need more exercise tests to find out how well your treatment is working. Each time testing is done, your doctor will compare your activity level with the previous one.
Pulmonary Hypertension Resource Guide
Pulmonary Hypertension (PH) is high blood pressure in the arteries to your lungs. It is a serious condition. If you have it, the blood vessels that carry blood from your heart to your lungs become hard and narrow. Your heart has to work harder to pump the blood through. Over time, your heart weakens and cannot do its job and you can develop heart disease.
Symptoms of Pulmonary Hypertension include the following:
- Shortness of breath during routine activity, such as climbing two flights of stairs
- Chest pain
- A racing heartbeat
- Pain on the upper right side of the abdomen
- Decreased appetite
As Pulmonary Hypertension worsens, you may find it hard to do any physical activities.
There are two main kinds of Pulmonary Hypertension. One runs in families or appears for no known reason. The other kind is related to another condition, usually heart or lung disease.
There is no cure for pulmonary hypertension. Treatments can control symptoms. They involve treating the heart or lung disease, medicines, oxygen, and sometimes lung transplantation.
How Is Pulmonary Hypertension Diagnosed?
Your doctor will diagnose pulmonary hypertension based on your medical and family histories, a physical exam, and results from tests and procedures performed.
Pulmonary hypertension can develop slowly. In fact, you may have it for years and not know it. This is because the condition has no early signs or symptoms.
When symptoms do occur, they're often like those of other heart and lung conditions, such as asthma. This makes pulmonary hypertension hard to diagnose.
Medical and Family Histories
Your doctor may ask about your signs and symptoms and how and when they began. Your doctor may also ask whether you have other medical conditions that can cause pulmonary hypertension.
Your doctor will want to know whether you have family members who have or had pulmonary hypertension. People who have a family history of PH are at higher risk for the condition.
During the physical exam, your doctor will listen to your heart and lungs with a stethoscope. The doctor will also check your ankles and legs for swelling and your lips and skin for a bluish color. These are signs of PAH (Pulmonary Hypertension).
Diagnostic Tests and Procedures for Pulmonary Hypertension
Your doctor may recommend tests and procedures to confirm a diagnosis of PH and to look for its underlying cause. Your doctor also will use test results to find out the severity of your pulmonary hypertension.
Tests / Procedures to Confirm Pulmonary Hypertension Diagnosis
Echo cardiography. Echo cardiography or echo, uses sound waves to create a moving picture of your heart. This test can estimate the pressure in your pulmonary arteries. Echo also can show the size and thickness of your right ventricle and how well it's working.
Chest x ray. A chest x ray takes pictures of the structures in your chest, such as your heart, lungs, and blood vessels. This test can show whether your pulmonary arteries and right ventricle are enlarged.
The pulmonary arteries and right ventricle may get larger if the right ventricle has to work hard to pump blood through the pulmonary arteries.
A chest x ray also may show signs of an underlying lung disease that's causing or contributing to PH.
EKG (electrocardiogram). An EKG is a simple, painless test that records the heart's electrical activity. This test also shows whether your heart's rhythm is steady or irregular. An EKG may show whether your right ventricle is enlarged or strained.
Right heart catheterization. This procedure measures the pressure in your pulmonary arteries. It also shows how well your heart is pumping blood to the rest of your body. Right heart catheterization can find any leaks between the left and right side of the heart.
During this procedure, a thin, flexible tube called a catheter is put into a blood vessel in your groin (upper thigh) or neck. The tube is threaded into the right side of your heart and into the pulmonary arteries. Through the tube, your doctor can do tests and treatments on your heart.
Tests for Underlying Cause of Pulmonary Hypertension
PH has many causes, so many tests may be needed to find its underlying cause.
Chest CT scan. A chest computed tomography scan, or chest CT scan, creates pictures of the structures inside your chest, such as your heart, lungs, and blood vessels. These pictures can show signs of pulmonary hypertension or a condition that may be causing PH.
Chest MRI. Chest magnetic resonance imaging, or chest MRI, shows how your right ventricle is working. The test also shows blood flow in your lungs. Chest MRI also can help detect signs of PH or an underlying condition causing PH.
Lung function tests. Lung function tests measure how much air you can breathe in and out, how fast you can breathe air out, and how well your lungs deliver oxygen to your blood. These tests can help detect a lung disease that may be causing PH.
Polysomnogram (PSG). This test records brain activity, eye movements, heart rate, and blood pressure while you sleep. A PSG also measures the level of oxygen in your blood. A low oxygen level during sleep is common in PH, and it can make the condition worse.
A PSG usually is done while you stay overnight at a sleep center. For more information about this test, go to the Diseases and Conditions Index Sleep Studies article.
Lung ventilation/perfusion (VQ) scan. A lung VQ scan measures air and blood flow in your lungs. This test can help detect blood clots in your lung's blood vessels.
Blood tests. Blood tests are used to rule out other diseases, such as HIV, liver disease, and autoimmune diseases (such as rheumatoid arthritis).
Treatment for Pulmonary Hypertension
Pulmonary hypertension (PH aka PAH) has no cure. However, treatment may help relieve symptoms and slow the progress of the disease.
PH or PAH is treated with medicines, procedures and other therapies. Treatment depends on what type of PH you have and severity.
Types of Pulmonary Hypertension
Group 1 Pulmonary Arterial Hypertension
Group 1 pulmonary arterial hypertension (PAH) includes PH that's inherited, that has no known cause, or that's caused by certain drugs or conditions. Treatments for group 1 PAH include medicines and medical procedures.
Medicines for PH
Your doctor may prescribe medicines to relax the blood vessels in your lungs and reduce excess cell growth in the blood vessels. As the blood vessels relax, more blood can flow through them.
Your doctor may prescribe medicines that are taken by mouth, inhaled, or injected.
Examples of medicines for group 1 PAH include:
- Phosphodiesterase-5 inhibitors, such as sildenafil
- Prostanoids, such as epoprostenol
- Endothelin receptor antagonists, such as bosentan and ambrisentan
- Calcium channel blockers, such as diltiazem
Your doctor may prescribe one or more of these medicines. To find out which of these medicines works best, you'll likely have an acute vasoreactivity test. This test shows how the pressure in your pulmonary arteries reacts to certain medicines. The test is done during right heart catheterization.
Medical and Surgical Procedures
If you have group 1 PAH, your doctor may recommend one or more of the following procedures.
Atrial septostomy (sep-TOS-toe-me). For this procedure, a thin, flexible tube called a catheter is put into a blood vessel in your leg and threaded to your heart. The tube is then put through the wall that separates your right and left atria (the upper chambers of your heart). This wall is called the septum.
A tiny balloon on the tip of the tube is inflated. This creates an opening between the atria. This procedure relieves the pressure in the right atria and increases blood flow. Atrial septostomy is rarely done in the United States.
Lung transplant. A lung transplant is surgery to replace a person's diseased lung with a healthy lung from a deceased donor. This procedure may be used for people who have severe lung disease that's causing PAH.
Heart–lung transplant. A heart–lung transplant is surgery in which both the heart and lung are replaced with healthy organs from a deceased donor.
Group 2 Pulmonary Hypertension
Conditions that affect the left side of the heart, such as mitral valve disease, can cause group 2 PH. Treating the underlying condition will help treat PH. Treatments may include lifestyle changes, medicines, and surgery.
Group 3 Pulmonary Hypertension
Lung diseases, such as COPD (chronic obstructive pulmonary disease) and interstitial lung disease, can cause group 3 PH. Certain sleep disorders, such as sleep apnea, also can cause group 3 PH.
If you have this type of PH, you may need oxygen therapy. This treatment raises the level of oxygen in your blood. You'll likely get the oxygen through soft, plastic prongs that fit into your nose. Oxygen therapy can be done at home or in a hospital.
Your doctor also may recommend other treatments if you have an underlying lung disease.
Group 4 Pulmonary Hypertension
Blood clots in the lungs or blood clotting disorders can cause group 4 PH. If you have this type of PH, your doctor will likely prescribe blood-thinning medicines. These medicines prevent clots from forming or getting larger.
Sometimes doctors use surgery to remove scarring in the pulmonary arteries due to old blood clots.
Group 5 Pulmonary Hypertension
Various diseases and conditions, such as thyroid disease and sarcoidosis, can cause group 5 PH. An object, such as a tumor, pressing on the pulmonary arteries also can cause group 5 PH.
Group 5 PH is treated by treating its cause.
All Types of Pulmonary Hypertension
Several treatments may be used for all types of PH. These treatments include:
- Diuretics, also called water pills. These medicines help reduce fluid buildup in your body, including swelling in your ankles and feet.
- Blood-thinning medicines. These medicines help prevent blood clots from forming or getting larger.
- Digoxin. This medicine helps the heart beat stronger and pump more blood. Digoxin sometimes is used to control the heart rate if abnormal heart rhythms, such as atrial fibrillation or atrial flutter, occur.
- Oxygen therapy. This treatment raises the level of oxygen in your blood.
- Physical activity. Regular activity may help improve your ability to be active. Talk with your doctor about a physical activity plan that's safe for you.
Research is ongoing for better PH treatments. These treatments offer hope for the future.
Living With Pulmonary Hypertension
Pulmonary hypertension has no cure. However, you can work with your doctor to manage your symptoms and slow the progress of the disease.
Ongoing Care for Pulmonary Hypertension
Follow your treatment plan as your doctor advises. Call your doctor if your PH symptoms worsen or change. The earlier symptoms are addressed, the easier it is to treat them.
Some symptoms, such as chest pain, may require emergency treatment. Ask your doctor when you should call him or her or seek emergency care.
Also, talk with your doctor before taking any over-the-counter medicines. Some medicines can make your PH worse or interfere with the medicines you're taking for PH. Ask your doctor whether you should get a pneumonia vaccine and a yearly flu shot.
You may have a complex schedule for taking medicines. Call your doctor or nurse if you're having problems with this schedule. Knowing the names of your medicines and how they work is helpful. Keep a list of your medicines with you. Don't stop or change medicines unless you talk with your doctor first.
Pay careful attention to your weight. You may want to keep a daily record of your weight. You should weigh yourself at the same time each day. If you notice a rapid weight gain (2 or more pounds in 1-day or 5 or more pounds in 1-week), call your doctor. This may be a sign that your PH is worsening.
Pregnancy is risky for women who have PH. Consider using birth control if there is a chance you may become pregnant. Ask your doctor which birth control methods are safe for you.
Lifestyle Changes for Pulmonary Hypertension
Making lifestyle changes can help you manage your symptoms. These changes will depend on the type of PH you have. Talk with your doctor about which lifestyle changes can help you.
If you smoke, quit. Smoking makes PH symptoms worse. Ask your doctor about programs and products that can help you quit. Also, avoid exposure to secondhand smoke.
For more information about how to quit smoking, go to the Diseases and Conditions Index Smoking and Your Heart article and the National Heart, Lung, and Blood Institute's (NHLBI's) "Your Guide to a Healthy Heart."
Although these resources focus on heart health, they both include general information about how to quit smoking.
Follow a Healthy Diet
Following a healthy diet and maintaining a healthy weight are part of a healthy lifestyle. A healthy diet includes a variety of fruits, vegetables, and whole grains. It also includes lean meats, poultry, fish, and fat-free or low-fat milk or milk products. A healthy diet also is low in saturated fat, trans fat, cholesterol, sodium (salt) and added sugar.
Talk with your doctor about whether you need to limit the amount of salt and fluids in your diet. Ask doctor whether you also need to regulate foods that contain vitamin K. These foods can affect how well blood-thinning medicines work. Vitamin K is found in green leafy vegetables and some oils, such as canola and soybean oil.
For more information about following a healthy diet, go to the NHLBI's - nhlbi.nih.gov website. These resources include general advice about healthy eating. The DASH eating plan focuses on reduced-sodium foods, which may be helpful if your doctor advises you to limit the salt in your diet.
Be Physically Active
Physical activity is an important part of a healthy lifestyle. Try to do physical activity, such as walking, regularly. This will keep your muscles strong and help you stay active. Ask your doctor how much activity is safe for you. Your doctor may tell you to limit or avoid certain activities, such as:
- Those that cause straining, such as lifting heavy objects or weights.
- Sitting in a hot tub or sauna or taking long baths. These activities can lower your blood pressure too much.
- Flying in an airplane or traveling to high-altitude areas. Your doctor may ask you to use extra oxygen during air travel.
Avoid activities that cause breathing problems, dizziness or chest pain. If you have any of these symptoms, seek care right away.
Emotional Issues / Support for Sufferers of Pulmonary Hypertension
Living with PH may cause fear, anxiety, depression and stress. You may worry about your medical condition, treatment, finances and other issues.
Talk about how you feel with your health care team. Talking to a professional counselor also can help. If you're very depressed, your doctor may recommend medicines or other treatments that can improve your quality of life.
Joining a patient support group may help you adjust to living with PH. You can see how other people with the same symptoms have coped with them. Talk with your doctor about local support groups or check with an area medical center.
Support from family and friends also can help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you.
The National Heart, Lung, and Blood Institute (NHLBI) is strongly committed to supporting research aimed at preventing and treating heart, lung, and blood diseases and conditions and sleep disorders.
NHLBI-supported research has led to many advances in medical knowledge and care. For example, this research has uncovered some of the causes of lung diseases and conditions, as well as ways to prevent or treat them.
Many more questions remain about lung diseases and conditions, including pulmonary hypertension (PH). The NHLBI continues to support research aimed at learning more about these diseases and conditions. For example, NHLBI-supported research on PH includes studies that explore:
- How often people who have sickle cell anemia develop PH
- Which genetic mutations (changes in the genes) cause PH; an example is the gene for bone morphogenetic receptor, type II (BMPR2)
- How certain medicines and therapies can help treat PH and improve quality of life for people who have the disease
- The effects of a rehabilitation exercise program on people with PH
- Whether inhaled nitric oxide (a gas) can help people with group 3 PH
Much of this research depends on the willingness of volunteers to take part in clinical trials. Clinical trials test new ways to prevent, diagnose, or treat various diseases and conditions.
For example, new treatments for a disease or condition (such as medicines, medical devices, surgeries, or procedures) are tested in volunteers who have the illness. Testing shows whether a treatment is safe and effective in humans before it is made available for widespread use.
By taking part in a clinical trial, you can gain access to new treatments before they're widely available. You also will have the support of a team of health care providers, who will likely monitor your health closely. Even if you don't directly benefit from the results of a clinical trial, the information gathered can help others and add to scientific knowledge.
If you volunteer for a clinical trial, the research will be explained to you in detail. You'll learn about treatments and tests you may receive, and the benefits and risks they may pose. You'll also be given a chance to ask questions about the research. This process is called informed consent.
If you agree to take part in the trial, you'll be asked to sign an informed consent form. This form is not a contract. You have the right to withdraw from a study at any time, for any reason. Also, you have the right to learn about new risks or findings that emerge during the trial.
For more information about clinical trials related to pulmonary hypertension, talk with your doctor.
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