Pulmonary hypertension

Symptoms

The symptoms of pulmonary hypertension develop slowly. You may not notice them for months or even years. Symptoms get worse as the disease continues.

Pulmonary hypertension symptoms include:

• Shortness of breath. It may first start during exercise and eventually happen at rest.
• Blue or gray skin. Depending on skin color, these changes may be harder or easier to see.
• Chest pressure or pain.
• Dizziness or fainting.
• Fast pulse or pounding heartbeat.
• Fatigue.
• Swelling in the ankles, legs and belly area.

These symptoms may be caused by many other health conditions. See a healthcare professional for an accurate diagnosis.

Risk factors

Pulmonary hypertension is usually seen in people ages 30 to 60. Growing older can increase the risk of developing Group 1 pulmonary hypertension, called pulmonary arterial hypertension (PAH). PAH from an unknown cause is more common in younger adults.

Other things that can raise the risk of pulmonary hypertension are:

• A family history of the condition.
• Being overweight.
• Smoking.
• Blood-clotting disorders or a family history of blood clots in the lungs.
• A history of being around asbestos.
• A heart condition present at birth, called a congenital heart defect.
• Living at an altitude of 8,000 feet (2,438 meters) or higher.
• Use of some medicines, including those used for weight loss.
• Illicit drugs such as cocaine or methamphetamine.

Diagnosis

Pulmonary hypertension is hard to diagnose early. It's not often found during a routine physical exam. Even when pulmonary hypertension is more advanced, its symptoms are similar to those of other heart and lung conditions.

To diagnose pulmonary hypertension, a healthcare professional examines you and asks about your symptoms. You are usually asked questions about your medical and family history.

Tests

Tests to diagnose pulmonary hypertension may include:

• Blood tests. Blood tests can help find the cause of pulmonary hypertension. The test also may help find complications of the disease.
• Chest X-ray. A chest X-ray is a picture of the heart, lungs and chest. It may be used to check for other lung conditions that can cause pulmonary hypertension.
• Electrocardiogram (ECG or EKG). This simple test records the electrical activity of the heart. It shows how the heart is beating.

• Echocardiogram. Sound waves create pictures of the beating heart. An echocardiogram shows how blood flows through the heart and heart valves. This test may be done to help diagnose pulmonary hypertension or to learn how treatments are working.

  Sometimes, an echocardiogram is done while exercising on a stationary bike or treadmill to learn how activity affects the heart. If you have this test, you may be asked to wear a mask that checks how well the heart and lungs use oxygen and carbon dioxide.

• Right heart catheterization. If an echocardiogram shows pulmonary hypertension, this test may be done to confirm the diagnosis.

  During this procedure, a doctor places a thin, flexible tube called a catheter into a blood vessel, usually in the neck. The tube is gently guided into the lower right heart chamber and the pulmonary artery. The doctor can then measure blood pressure in the main pulmonary arteries and the right ventricle.

Other tests may be done to check the condition of the lungs and pulmonary arteries. The following tests may give more information about the cause of pulmonary hypertension:

• Exercise stress tests. These tests often involve walking on a treadmill or riding a stationary bike while the heartbeat is watched. They can show how the heart reacts to exercise.

• Computerized tomography (CT) scan. This test uses X-rays to make pictures of specific parts of the body. Dye called contrast may be given into a vein to help the blood vessels show up more clearly on the images.

  A heart CT scan, called a cardiac CT scan, can show the size of the heart and any blockages in the pulmonary arteries. It can help diagnose lung diseases that might lead to pulmonary hypertension. Examples are COPD or pulmonary fibrosis.

• Magnetic resonance imaging (MRI). This test uses magnetic fields and radio waves to make detailed pictures of the heart. It can show blood flow in the pulmonary arteries. The test may be done to learn how well the right lower heart chamber is working.
• Lung function test. For this test, you blow into a special device. The device measures how much air the lungs can hold. It shows how air flows in and out of the lungs.
• Sleep study. A sleep study measures brain activity, heart rate, blood pressure, oxygen levels and other things as you sleep. The test can help diagnose sleep apnea, which can cause pulmonary hypertension.
• Ventilation/perfusion (V/Q) scan. In this test, a radioactive tracer is given through a vein (IV). The tracer shows how blood flows. You also may breathe in a tracer that shows airflow to the lungs. A V/Q scan can tell whether blood clots are causing symptoms of pulmonary hypertension.
• Lung biopsy. Rarely, a sample of tissue may be taken from the lung to check for a possible cause of pulmonary hypertension.

Genetic testing

Screening for gene changes that cause pulmonary hypertension may be recommended. If you have these gene changes, other family members may need to be screened too.

Pulmonary hypertension functional classification

Once a diagnosis of pulmonary hypertension is confirmed, the condition is classified according to how the symptoms affect you and your ability to do everyday tasks.

Pulmonary hypertension may fall into one of the following groups:

• Class I. Pulmonary hypertension is diagnosed, but there are no symptoms during rest or exercise.
• Class II. There are no symptoms at rest. Everyday chores or activities such as going to work or the grocery store may cause some shortness of breath or mild chest pain. There's a slight limitation of physical activity.
• Class III. It's comfortable at rest, but doing simple tasks such as bathing, dressing or preparing meals causes fatigue, shortness of breath and chest pain. The ability to do physical activity becomes very limited.
• Class IV. Symptoms occur at rest and during physical activity. Any type of activity causes increasing discomfort.

Your healthcare team may use a risk calculator that looks at symptoms and test results to understand what type of treatment is needed. This is called pulmonary hypertension risk stratification.

Lifestyle and home remedies

Lifestyle changes may help improve pulmonary hypertension symptoms. Try these tips:

• Eat healthy. Eat a healthy diet rich in whole grains, fruits and vegetables, lean meats, and low-fat dairy products. Try to stay away from saturated fat, trans fat and cholesterol. Use less salt.
• Stay as active as possible and manage weight. Even mild forms of activity might be too exhausting for some people who have pulmonary hypertension. For others, moderate exercise, such as walking, might be helpful — especially when done during oxygen therapy. Your healthcare team can help you plan an appropriate exercise program.
• Don't smoke. If you smoke, quit. If you need help, ask your healthcare team for treatment that can help. Avoid secondhand smoke too, if possible.
• Get plenty of rest. Resting can reduce tiredness related to pulmonary hypertension.
• Avoid high altitudes. High altitudes can make pulmonary hypertension worse. If you live at an altitude of 8,000 feet (2,438 meters) or higher, you might be told to consider moving to a lower altitude.
• Avoid activities that can lower blood pressure a lot. These include sitting in a hot tub or sauna or taking long hot baths or showers. Such activities lower blood pressure and can cause fainting. Also, do not do activities that cause a lot of straining, such as lifting heavy objects or weights.
• Tell your healthcare team about the medicines you take. Some medicines can make pulmonary hypertension worse or affect its treatment.
• Get regular health checkups. Tell your healthcare team about any new or worsening symptoms or medicine side effects. If pulmonary hypertension affects your quality of life, ask about treatments that could help.
• Get recommended vaccines. Respiratory infections can cause serious health concerns for people with pulmonary hypertension. Ask your healthcare team which vaccines you need to prevent common viral infections.
• Talk to a healthcare professional before becoming pregnant. Pulmonary hypertension can cause serious complications for the pregnant person and unborn baby, also called a fetus. Birth control pills can increase the risk of blood clots. Talk to your healthcare team about other birth control options.

Preparing for an appointment

If you think that you are at risk of or that you might have pulmonary hypertension, make an appointment for a health checkup.

There's often a lot to discuss at your appointment, so it's a good idea to be prepared. Here's some information to help you get ready for your appointment.

What you can do

• Be aware of any preappointment restrictions. When you make your appointment, ask if there is anything you need to do before your checkup. For example, you might be told not to eat or drink before some medical tests.
• Write down any symptoms you're having, including any that might not seem related to pulmonary hypertension. Try to remember when they began. Be specific, such as days, weeks and months.
• Make a list of important personal information. Include any family history of pulmonary hypertension, lung disease, heart disease, stroke, high blood pressure or diabetes. Also list any major stresses or recent life changes.
• Make a list of all medicines that you take. Also include vitamins, herbal products, supplements and any medicines bought without a prescription.
• Take someone with you, if possible. Someone who goes with you can help you remember information you're given.
• Be prepared to discuss your diet and exercise habits. If you don't already follow a diet or exercise routine, talk to your healthcare team about any challenges you might face in getting started.
• Make a list of questions to ask your healthcare team. List your questions from most important to least important in case time runs out.

For pulmonary hypertension, some questions to ask your healthcare team are:

• What is the likely cause of my symptoms or condition?
• What are other possible causes?
• What tests do I need?
• What treatment do you recommend?
• What are the other treatment options?
• Is there a generic form of the medicine you're prescribing?
• What's an appropriate level of physical activity?
• Are there any restrictions that I need to follow?
• How often do I need health checkups?
• I have other health conditions. How can I best manage them together?
• Should I see a specialist?
• Is there any information that I can take home? What websites do you suggest?

Don't hesitate to ask other questions.

What to expect from your doctor

Your healthcare team may ask you many questions. Being ready to answer them might give you more time to discuss any concerns. You may be asked:

• When did you first begin having symptoms?
• Do you always have symptoms, or do they come and go?
• On a scale of 1 to 10, with 10 being the worst, how bad are your symptoms?
• What, if anything, seems to make your symptoms better?
• What, if anything, seems to make your symptoms worse?

What you can do in the meantime

It's never too late to make healthy lifestyle changes. Do not smoke, eat less salt, and choose nutritious foods. These changes may help prevent pulmonary hypertension from getting worse.