Pulmonary Hypertension (PH)

What is pulmonary hypertension?

Pulmonary hypertension (PH) is a general diagnosis that means you have high blood pressure in your pulmonary arteries. These are the blood vessels that carry oxygen-poor blood from your heart to your lungs.

Pulmonary hypertension has many different causes. It’s usually a complication of heart disease or lung disease. But many other diseases and environmental factors can raise your risk for PH.

Pulmonary hypertension is dangerous because it disrupts the flow of blood through your heart and lungs. High blood pressure in your pulmonary arteries causes these arteries to become narrow. As a result, your heart must work harder to pump oxygen-poor blood to your lungs.

Over time, PH damages your heart and causes problems throughout your body. It can be fatal without treatment.

How does pulmonary hypertension affect my body?

Pulmonary hypertension can cause serious problems in your body, including:

• Anemia.
• Arrhythmias.
• Blood clots in your pulmonary arteries.
• Pericardial effusion.

PH is dangerous for people who are pregnant. It can cause complications for both the birthing parent and fetus.

Without treatment, pulmonary hypertension can overtax your heart and eventually be fatal. High blood pressure in your pulmonary arteries forces your heart to work harder to send oxygen-poor blood to your lungs. Your right ventricle (lower right chamber) is responsible for pumping this blood to your lungs. So, over time, PH causes your right ventricle to get bigger due to the extra work. This condition (right ventricular hypertrophy) can lead to right-sided heart failure.

Right-sided heart failure has a ripple effect throughout your body. It can disrupt the normal workings of many organs and systems.

Because pulmonary hypertension can affect your entire body, it’s essential that you’re diagnosed and treated as early as possible. Your provider will prescribe treatment based on what’s causing your PH. No matter the cause, untreated PH is life-threatening.

What are the different types of pulmonary hypertension?

The World Health Organization (WHO) divides pulmonary hypertension into five groups based on its cause.

• Group 1 PH due to pulmonary arterial hypertension (PAH). PAH has many different causes, ranging from underlying diseases to certain drugs. PAH makes your pulmonary arteries become narrow, thick or stiff. Less blood can flow through, which raises the pressure in your pulmonary arteries.
• Group 2 PH due to left-sided heart disease. The left side of your heart pumps out blood to your entire body. If there’s a problem on this side of your heart, it affects the right side of your heart and your entire pulmonary circuit. Blood backs up in your heart, raising the pressure in your pulmonary arteries.
• Group 3 PH due to lung disease or hypoxia. Certain lung problems cause the arteries in your lungs to tighten. Less blood can flow through your lungs, raising the pressure in your pulmonary arteries.
• Group 4 PH due to blockages in your lungs. Blood clots or scars from blood clots prevent your blood from flowing normally through your lungs. This puts more stress on the right side of your heart and raises pulmonary blood pressure.
• Group 5 PH due to other disorders. PH occurs along with other conditions like blood disorders and metabolic disorders. The exact mechanisms for how the condition triggers PH aren’t always clear.

Who does pulmonary hypertension affect?

Pulmonary hypertension can affect adults at any age. It commonly affects people who have heart or lung conditions. It’s also more common among people with other medical conditions. PH affects:

• Nearly 100% of people with severe mitral valve disease.
• About 65% of people with aortic valve disease.
• Up to 30% of people with scleroderma.
• About 20% to 40% of people with sickle cell disease.
• About 1 in 200 people with HIV.

PH usually affects adults. But rarely, it can affect newborns. This is called persistent pulmonary hypertension of the newborn (PPHN). Infants with this condition may need treatment in the intensive care unit.

How common is pulmonary hypertension?

Some types of PH are rare, such as pulmonary arterial hypertension (PAH) and PH caused by blood clots. But other types are much more common, especially PH caused by heart or lung problems.

We don’t know exactly how many people around the world have pulmonary hypertension. But some estimates show PH may affect 1 in 100 people. This means 50 million to 70 million people are living with PH.

PH is even more common among older adults. Around the world, about 1 in 10 adults over age 65 have PH.

Researchers believe the number of people diagnosed with PH will rise in the next few decades.

What are the first symptoms of pulmonary hypertension?

The first symptom of pulmonary hypertension is shortness of breath during your daily activities. These may include climbing stairs or grocery shopping. You may also feel short of breath when you exercise.

At the start of pulmonary hypertension, you may not have any symptoms. When you do start to notice symptoms, they may be mild. But PH symptoms get worse over time, making it harder for you to do your usual activities.

What are the later symptoms of pulmonary hypertension?

As PH progresses, you’ll feel shortness of breath even when you’re not moving around. Other symptoms include:

• Bluish color on your skin or lips.
• Chest pain or pressure.
• Dizziness or fainting.
• Fatigue.
• Feeling less hungry than usual.
• Pain in the upper right side of your tummy.
• Racing heartbeat.
• Swelling (edema) in your ankles, legs or tummy.

PH symptoms make it hard for you to exercise or carry out your normal daily routine.

What are the stages of pulmonary hypertension?

There are four main stages of pulmonary hypertension. The World Health Organization (WHO) calls these “functional classes.” They’re based on the symptoms you feel and refer to how well you can carry out your daily activities. As PH gets worse, the symptoms become more noticeable and more disruptive to your daily life.

• Class 1: You don’t have any symptoms.
• Class 2: You don’t have symptoms when you’re resting. But you feel some discomfort or shortness of breath during some routine activities. These include household chores and climbing stairs.
• Class 3: You may still feel fine when you’re resting. But it’s now much harder to do normal tasks because you feel tired or short of breath.
• Class 4: You have symptoms even when you’re resting. The symptoms get worse when you try to do any normal task.

What causes pulmonary hypertension?

Causes of pulmonary hypertension vary widely depending on the type of PH you have. They include a range of diseases and underlying conditions as well as environmental exposures (toxins and drugs).

Group 1 PH due to pulmonary arterial hypertension (PAH)

There are many causes of pulmonary arterial hypertension. They include:

• Congenital heart disease.
• Diet pills like “fen-phen” (which can cause PAH years after use).
• Genetic mutations.
• Glycogen storage diseases.
• HIV.
• Liver disease.
• Lupus.
• Portal hypertension.
• Pulmonary capillary hemangiomatosis.
• Pulmonary veno-occlusive disease.
• Schistosomiasis.
• Scleroderma.
• Recreational drugs like methamphetamine.

Some people develop PAH without any clear cause. These cases are known as “idiopathic.”

Group 2 PH due to left-sided heart disease

Heart problems are a common cause of pulmonary hypertension. Because the left and right sides of your heart work together, a problem with the left side also affects the right side. The right side of your heart is responsible for pumping blood into your pulmonary arteries. So, left-sided heart problems cause a chain reaction that affects the rest of your heart, your pulmonary arteries and your lungs.

Left-sided heart problems that can cause pulmonary hypertension include:

• Aortic valve disease. Your aortic valve connects your left ventricle (your heart’s main pumping chamber) to your aorta. Your aorta is the largest blood vessel in your body, and it’s responsible for sending oxygen-rich blood out to the rest of your body. With aortic valve disease, your valve may be leaky (regurgitation) or narrow (stenosis). Other problems like congenital heart disease and endocarditis can also affect your aortic valve.
• Left-sided heart failure. This means the left side of your heart can’t pump blood as it should. Your left ventricle may be too weak, or it may be too stiff. Either way, not enough oxygen-rich blood can reach your body’s organs and tissues. Over time, left-sided heart failure can lead to right-sided heart failure and other complications.
• Left ventricular hypertrophy. This is a thickening of the muscular walls of your left ventricle that makes your heart less efficient at pumping blood. It’s usually caused by long-term hypertension or other heart problems like arrhythmias.
• Mitral valve disease. Your mitral valve is the door that lets blood flow from your left atrium to your left ventricle. It may become too stretchy (prolapse), leading to regurgitation. It may also become too narrow. Over time, mitral valve disease strains and damages your heart.

Group 3 PH due to lung disease or hypoxia

Lung problems are another common cause of pulmonary hypertension. Some people with left-sided heart disease also have lung disease or a lack of oxygen (hypoxia).

Lung issues that can cause pulmonary hypertension include:

• Chronic obstructive pulmonary disease (COPD). This is a group of diseases that includes chronic bronchitis and emphysema. COPD makes it harder for you to breathe.
• Interstitial lung disease. This means you have fibrosis, or scarring, in your lung tissue. The scarring leads to breathing problems and coughing.
• Obstructive sleep apnea. This condition causes your airway to become partly or completely blocked, over and over again, as you sleep. As a result, less oxygen reaches your organs.

Group 4 PH due to blockages in your lungs

This form of PH is usually caused by chronic thromboembolic pulmonary hypertension (CTEPH). CTEPH is a condition that involves blood clots and scarring in the arteries in your lungs.

A pulmonary embolism is a blood clot that travels from somewhere else in your body and gets stuck in a blood vessel in your lungs. These blood clots are often treatable, but they may leave behind scar tissue.

These scars can make it harder for blood to flow through your pulmonary arteries. As a result, the blood pressure in your pulmonary arteries goes up.

Group 5 PH due to other disorders

Many other conditions cause pulmonary hypertension in ways that scientists don’t yet understand. Scientists don’t know the exact mechanisms that cause these conditions to trigger PH. What they do know is that there’s an association between these conditions and PH. That means people with these conditions may face a higher risk of developing PH. Such conditions include:

• Gaucher disease: A metabolic disorder that affects your organs and bones.
• Kidney disease: Problems with the function of your kidneys.
• Langerhans cell histiocytosis: A disorder that, in adults, causes scarring and cysts in your lungs.
• Sarcoidosis: An inflammatory disease that usually affects your lungs and lymph glands.
• Thyroid disease: Disorders that affect your thyroid’s production of hormones.
• Tumors: Cancerous or benign tumors may put pressure on blood vessels in your lungs.

How is pulmonary hypertension diagnosed?

Your provider will perform a physical exam and run tests to reach a pulmonary hypertension diagnosis.

First, you’ll have a physical exam to check for signs of pulmonary hypertension as well as other heart or lung issues. During this exam, your provider will:

• Ask you questions about your health and your medical history.
• Ask about your symptoms.
• Check the size of the veins in your neck. Bulging neck veins (jugular venous distention) could be a sign of right-sided heart failure.
• Check the size of your liver by feeling the upper right area of your tummy.
• Listen to your heart and lungs with a stethoscope.
• Look at your belly, ankles and legs for edema.
• Measure your blood pressure.
• Measure the oxygen level in your blood using a pulse oximeter.

Pulmonary hypertension can be difficult to diagnose since many signs of PH are similar to those of other conditions. So, after your physical exam, your provider may run some tests to get more information.

Your provider may also refer you to a pulmonologist or cardiologist.

What tests diagnose pulmonary hypertension?

Your provider may use several different tests for different purposes.

These tests measure the blood pressure in your pulmonary arteries:

• Right heart catheterization: This test is also called pulmonary artery catheterization. It measures the pressure inside your pulmonary arteries and checks how much blood your heart can pump per minute.
• Doppler echocardiogram: A Doppler echo uses sound waves to show how your right ventricle is working. It also measures blood flow through your heart valves. It allows your provider to calculate your systolic pulmonary artery pressure.

These tests look for the underlying cause of pulmonary hypertension:

• Blood tests: Check for a range of issues related to organ function, hormone levels and infections. Specific blood tests include a complete metabolic panel and a complete blood count.
• Chest CT scan: Looks for blood clots and other lung conditions that may be causing your pulmonary hypertension or making it worse.
• Chest X-ray: Shows if your right ventricle or pulmonary arteries are bigger than they should be.
• Polysomnogram (PSG): This overnight sleep test checks if you have sleep apnea.
• Pulmonary ventilation/perfusion (VQ) scan: Looks for blood clots in your lungs.

Your provider may also perform a six-minute walk test. This test shows how much exercise you can handle and how much oxygen is circulating in your blood as you exercise. The results indicate if your pulmonary hypertension is mild or severe.

What is the treatment for pulmonary hypertension?

Pulmonary hypertension treatment depends on the type of PH you have and your other medical conditions. Your healthcare team will tailor treatment to your individual needs.

Right now, only two types of PH can be treated directly:

• Pulmonary artery hypertension (PAH).
• Chronic thromboembolic pulmonary hypertension (CTEPH).

Treatment for other types of PH involves managing the underlying medical conditions.

Treatment for pulmonary arterial hypertension (PAH) includes:

• Calcium channel blockers. These medications can help lower the blood pressure in your pulmonary arteries and throughout your body.
• Diuretics. These “water pills” help your body clear out extra fluid.
• Oxygen therapy. You may need this treatment if you don’t have enough oxygen in your blood.
• Pulmonary vasodilators. These medications help your pulmonary arteries relax and open up better. This improves blood flow and lowers the strain on your heart.

Treatment for CTEPH includes:

• Anticoagulants. These medicines help prevent blood clots.
• Balloon atrial septostomy (BAS). This procedure is typically used for babies with critical heart defects. However, it’s also used for adults with pulmonary hypertension. It’s a bridge that helps keep you stable as you wait for a lung transplant.
• Balloon pulmonary angioplasty (BPA). This catheter-based procedure uses a balloon to widen your pulmonary artery. It’s usually done if you can’t have open surgery.
• Medication. A soluble guanylate cyclase stimulator (SGCS) may help slow down the disease progression.
• Pulmonary endarterectomy (PEA). This surgery removes blood clots from your lungs. It’s currently the only possible cure for pulmonary hypertension, and it’s only for people with CTEPH.

Treatment for PH caused by heart or lung problems focuses on managing the underlying conditions. Because so many different heart and lung conditions cause PH, treatment plans can be vastly different from person to person. Talk with your provider about what’s best for you. In general, your provider may recommend:

• Dietary changes.
• Lifestyle changes.
• Medication to manage problems like hypertension or heart failure.
• Oxygen therapy.
• Surgery, such as heart valve repair.

Treatments for PH caused by other medical conditions (WHO Group 5) are still evolving. Your provider will work with you to determine the best care plan.

A last resort option for some people with severe pulmonary hypertension is a lung transplant.

What medications treat pulmonary hypertension?

Pulmonary vasodilators are medications that treat PAH and CTEPH. They can’t be used for other types of PH, including those caused by underlying heart or lung issues.

Pulmonary vasodilators help your pulmonary arteries relax. This lowers blood pressure and eases the load on the right side of your heart.

People with PH may take a variety of other medications based on their underlying conditions.

How should I manage my pulmonary hypertension medications?

You may be taking many different medications to treat PH and other medical conditions. It can be a lot to keep track of. Here’s some advice.

• Know the names of all your medications, and know how they work. Keep a list with you at all times.
• Take your medications at the same time every day. If you forget a dose, don’t take two doses to make up for the dose you missed. Call your provider and ask what to do.
• Don’t take any over-the-counter medications unless you ask your provider first. Some cold and flu medicines and pain relievers can cause problems for people with heart failure.
• Avoid any over-the-counter medications that have cautions for people with high blood pressure.
• Don’t stop taking any medication or make any changes to your medications unless you first talk with your provider.
• Avoid herbal supplements including herbal tea. Herbal products may interact with some of your medications.

What raises a person’s risk of developing pulmonary hypertension?

Risk factors for developing pulmonary hypertension include:

• Exposure to asbestos.
• Family history of blood clots.
• Family history of pulmonary hypertension.
• Living at high altitudes.
• Smoking and using tobacco products.
• Use of diet medications such as “fen-phen” (dexfenfluramine and phentermine).
• Use of some prescription medications that treat cancer and depression.
• Use of recreational drugs.

Certain medical conditions also raise your risk. These include:

• Blood clots in your pulmonary arteries.
• Connective tissue disease.
• Down syndrome.
• Gaucher disease.
• Heart disease.
• HIV.
• Liver disease.
• Lung disease.

Talk with your provider about your risk factors and what you can do to lower your risk.

How can I prevent pulmonary hypertension?

It’s not always possible to prevent pulmonary hypertension. Some risk factors are out of your control. If you have risk factors, your provider may recommend preventive screenings to check your heart and lung function.

Doing whatever you can to prevent or manage other medical conditions can help lower your risk of pulmonary hypertension. Steps you can take include:

• Create an exercise plan. Ask your provider what exercises are safe for you.
• Follow a heart-healthy diet. Avoid processed foods, fast food and other foods high in salt and saturated fat.
• Quit smoking and stop using tobacco. Smoking and tobacco use are top risk factors for heart and lung problems. Quitting isn’t easy, especially if you’ve been smoking or using tobacco for a long time. But your provider can help provide resources. Support groups may also help.
• Take medications for blood pressure and other conditions as prescribed.

What is the outlook for people with pulmonary hypertension?

The outlook for people with pulmonary hypertension depends on:

• The cause of PH.
• How early it’s diagnosed.
• The severity of symptoms.
• Associated medical conditions.

The outlook for each person is different. Talk with your provider to learn more about your prognosis and how to manage your condition.

Can pulmonary hypertension be cured?

Most cases of pulmonary hypertension can’t be cured. Your provider may prescribe medications to:

• Ease your symptoms.
• Improve your quality of life.
• Slow down the progression of the disease.

Your provider may also recommend lifestyle changes.

However, surgery can cure some people with chronic thromboembolic pulmonary hypertension (CTEPH).

What is the life expectancy for people with pulmonary hypertension?

The life expectancy varies from person to person. It depends how quickly you’re diagnosed and what other medical conditions you have. Talk with your provider about what you can expect in your individual situation.

Pulmonary hypertension is a progressive disease. That means it gets worse over time. It progresses more quickly in some people than in others. Treatment can improve your chances of surviving pulmonary hypertension for many years.

Is pulmonary hypertension fatal?

Without treatment, pulmonary hypertension leads to right-sided heart failure and, ultimately, death. Treatment can help you live longer and give you a better quality of life.

What follow-up care do I need?

Talk with your provider about how to manage and monitor your condition. Your provider will tell you when you need to come in for checkups and tests. Be sure to tell your provider about any new or changing symptoms.

Also, talk with your provider about:

• Birth control options and whether it’s safe to become pregnant. Pregnancy is risky for people with pulmonary hypertension.
• Creating an emergency kit. People with pulmonary hypertension need certain supplies and information with them at all times. Ask your provider what you should include in your emergency kit.
• Exercise. Ask what’s safe for you and how best to add exercise into your daily life. Ask what exercises to avoid.
• Seasonal vaccines, including for flu and pneumonia. Your provider will let you know which vaccines you should receive.
• Support groups or counseling as you adjust to the “new normal” of life with pulmonary hypertension. A medical diagnosis brings many emotions. You don’t have to carry them alone.
• Travel plans. You may need to take certain precautions if flying on an airplane. You may also need to be careful when traveling to high altitudes. Talk with your provider about how to prepare for travel and what to bring with you.

What dietary changes should I make?

Your provider will give you specific recommendations. One key step involves reducing your sodium intake. This means:

• Avoid adding salt at the table or using “seasoning salt.”
• Avoid smoked, cured, salted and canned meat products.
• Buy foods that are “low sodium” or “low salt.”
• Limit fast foods and prepared foods.

Other dietary changes include:

• Eat foods high in fiber (like whole grains, bran, fruits and vegetables).
• Eat foods high in potassium (like dried fruits, bananas and oranges).
• Eat foods high in magnesium (like peanuts, tofu and broccoli).
• Limit foods that contain refined sugar, saturated fat and cholesterol.

When should I call my healthcare provider?

Call your provider if you’re having problems with:

• A fast heart rate (120 beats per minute).
• A respiratory infection or cough that’s getting worse.
• Constantly feeling dizzy or lightheaded.
• Episodes of chest pain or discomfort with physical activity.
• Extreme fatigue or decreased ability to do your normal activities.
• Nausea or lack of appetite.
• Restlessness or confusion.
• Shortness of breath that’s gotten worse, especially if you wake up feeling short of breath.
• Swelling in your ankles, legs or tummy that’s gotten worse.
• Trouble breathing with regular activities or at rest.
• Weight gain (2 pounds in one day or 5 pounds in one week).

When should I go to the ER?

Go to the emergency department or call your local emergency number if you have:

• A fast heart rate (120-150 beats per minute) that won’t go down.
• Fainting spells with loss of consciousness.
• Hickman catheter complications with intravenous prostacyclins. These include infection, catheter displacement, solution leak, bleeding and IV pump malfunction.
• Shortness of breath that doesn’t go away when you rest.
• Sudden and severe chest pain.
• Sudden and severe headache.
• Sudden weakness or paralysis in your arms or legs.

A note from Cleveland Clinic

A pulmonary hypertension diagnosis can cause a range of emotions. It takes time to process the diagnosis, learn what’s going on inside your body and figure out how to move forward. Work with your provider to get the resources you need. Involve your family and friends in your lifestyle changes. Educate them on your condition. And most of all, know it’s OK to ask for help and lean on others as you adjust to your new normal.