Pulmonary Hypertension

Also known as pulmonary arterial hypertension (PAH)
Pulmonary hypertension occurs when the pressure in the blood vessels that carry blood from your heart to your lungs is higher than normal. One type of pulmonary hypertension is pulmonary arterial hypertension (PAH). Pulmonary hypertension can happen on its own or be caused by another disease or condition. In the United States, the most common cause of pulmonary hypertension is left heart disease. Other conditions that can cause pulmonary hypertension include sickle cell disease; pulmonary embolus, which is a type of venous thromboembolism; and chronic obstructive pulmonary disease (COPD).

The increased pressure in the blood vessels of the lungs means that your heart has to work harder to pump blood into the lungs. This can cause symptoms such as shortness of breath, chest pain, and lightheadedness. If left untreated, the increased pressure can damage your heart. This may lead to serious or life-threatening complications, such as heart failure or arrhythmias, which are irregular heart rhythms.

Explore this Health Topic to learn more about pulmonary hypertension, our role in research and clinical trials to improve health, and where to find more information.

Causes

Your genes or other medical conditions can cause pulmonary hypertension. Certain medical conditions can damage, change, or block the blood vessels of the pulmonary arteries. The cause of pulmonary hypertension is not always clear.

To understand pulmonary hypertension, it is helpful to understand the flow of blood through the heart and lungs. The right side of your heart receives oxygen-poor blood from your body’s tissues. The pulmonary arteries connect your right heart and lungs. The heart pumps blood through the pulmonary arteries to the lungs to become oxygen-rich blood. The force or pressure of the blood against the walls of the pulmonary arteries is called the pulmonary pressure.

To learn more, visit How the Lungs Work and How the Heart Works.

 

Genes

Gene mutations are found in some people who have a family history of pulmonary arterial hypertension. Mutations are also found often in patients who do not have a family history.

Medical conditions

Many medical conditions can cause pulmonary hypertension. One type of pulmonary hypertension—called pulmonary arterial hypertension—is caused by conditions that result in narrowing of the pulmonary arteries themselves, such as scleroderma or HIV. Narrowed blood vessels can increase blood pressure in the lungs.

Medical conditions that can cause pulmonary hypertension include:

Look for

  • Treatment will discuss medicines and procedures that your doctors may recommend if you are diagnosed with pulmonary hypertension.

Risk Factors

You may have an increased risk for pulmonary hypertension because of your age, environment, family history and genetics, lifestyle habits, medicines you are taking, other medical conditions, or sex.

Age

Your risk of pulmonary hypertension goes up as you get older, although it may occur at any age. The condition is typically diagnosed between ages 30 and 60.

Environment

You may be at an increased risk of pulmonary hypertension if you have or are exposed to the following:

  • Asbestos or silica
  • Infection caused by parasites such as schistosomiasis or Echinococcus, which are tapeworms

Family history and genetics

Certain genetic disorders, such as Down syndrome, congenital heart disease, and Gaucher disease, can increase your risk of developing pulmonary hypertension.

A family history of blood clots or pulmonary embolism also increases your risk of developing pulmonary hypertension.

Lifestyle habits

Unhealthy lifestyle habits can increase the risk of pulmonary hypertension. These habits include:

  • Illegal drugs, such as cocaine and amphetamines
  • Smoking

Medicines

Some medicines may increase your risk of pulmonary hypertension, including:

  • Chemotherapy medicines to treat cancer, such as dasatinib, mitomycin C, and cyclophosphamide
  • Selective serotonin reuptake inhibitors (SSRIs) to treat depression and anxiety. SSRIs may cause pulmonary arterial hypertension in newborns whose mothers have taken these medicines during pregnancy.
  • Weight-loss drugs such as fenfluramine and dexfenfluramine, which are no longer approved for weight loss in the United States

Other medical conditions

Certain medical conditions may increase your risk of developing pulmonary hypertension:

  • Blood clotting disorders, such as blood clots in the lungs, a higher-than-normal platelet count in your blood, and conditions that make your blood more likely to clot, such as protein S and C deficiency, factor V Leiden thrombophilia, antithrombin III deficiency, and antiphospholipid syndrome
  • Chronic kidney disease
  • Diseases that change the structure of the chest wall, such as scoliosis
  • Infections such as hepatitis B or C
  • Liver disease such as cirrhosis
  • Surgical removal of the spleen
  • Thyroid diseases

Sex

Pulmonary hypertension is more common in women than in men. Pulmonary hypertension with certain types of heart failure is also more common in women.

Screening and Prevention

To screen for pulmonary hypertension, your doctor will determine whether you have any known risk factors and may have you undergo screening tests. Screening is not usually performed unless you have known risk factors such as scleroderma. Your doctor may recommend prevention strategies to help you lower your risk of developing pulmonary hypertension.

Tests to screen for pulmonary hypertension

Based on your symptoms or risk factors, your doctor may recommend the following tests to screen for changes in the heart or lungs that may be related to pulmonary hypertension.

  • Echocardiography to look at your heart’s function and structure and estimate pulmonary artery pressure
  • Electrocardiography (ECG or EKG) to look for signs of changes in your heart or abnormal rhythms in your heart’s electrical activity.
  • Pulmonary function tests to look for changes in lung function for conditions such as systemic sclerosis, COPD, or interstitial lung diseases

Based on the results of these screening tests, your doctor may do follow-up tests to see whether you have higher-than-normal pressures in the pulmonary arteries. These other tests can help diagnose pulmonary hypertension.

Prevention strategies

To help prevent pulmonary hypertension, your doctor may recommend controlling certain medical conditions, avoiding certain medicines or illegal drugs, and protecting yourself against environmental hazards that are risk factors.

Look for

  • Diagnosis will discuss tests and procedures that your doctor may use to diagnose pulmonary hypertension.
  • Living With will discuss what your doctor may recommend to prevent your pulmonary hypertension from recurring, getting worse, or causing complications.
  • Research for Your Health will discuss how we are using current research and advancing research to prevent pulmonary hypertension.
  • Participate in NHLBI Clinical Trials will discuss our open and enrolling clinical studies that are investigating prevention strategies for pulmonary hypertension.

Signs, Symptoms, and Complications

Signs and symptoms of pulmonary hypertension are sometimes hard to recognize, because they are similar to those of other medical conditions. People may have symptoms for years before being diagnosed with pulmonary hypertension. These symptoms may get worse over time and could eventually lead to serious complications such as right heart failure.

Signs and symptoms

Signs and symptoms of pulmonary hypertension may include the following:

  • Chest pain
  • Cough that is dry or may produce blood
  • Fatigue
  • Hoarseness
  • Light-headedness, fainting, or dizziness
  • Nausea and vomiting
  • Shortness of breath, first with physical activity and then without it as the disease gets worse
  • Swelling of your abdomen, legs, or feet caused by fluid buildup
  • Weakness
  • Wheezing

Complications

Complications of pulmonary hypertension may include the following:

  • Anemia
  • Arrhythmias and bundle branch blocks of the heart
  • Blood clots in the pulmonary arteries
  • Bleeding in the lungs, which may be life-threatening
  • Heart failure, especially right ventricular failure
  • Liver damage from increased pressure in the right heart
  • Pericardial effusion, which is a collection of fluid in the sac-like structure around the heart
  • Pregnancy complications that can be life-threatening for the mother and baby

Look for

  • Diagnosis will discuss tests and procedures used to detect signs of pulmonary hypertension and help rule out other conditions that may mimic pulmonary hypertension.
  • Treatment will discuss treatment-related complications or side effects.

Diagnosis

To diagnose pulmonary hypertension, your doctor may ask you questions about your medical history and do a physical exam. Your doctor may also test you for pulmonary hypertension based on your signs and symptoms and risk factors. A diagnosis of pulmonary hypertension will be made if tests show higher-than-normal pressure in the pulmonary arteries.

Confirming high pressures in the pulmonary arteries

Normal pressure in the pulmonary arteries is between 11 and 20 millimeters of mercury (mm Hg) when measured by cardiac catheterization. Your doctor may perform the following tests to confirm high pressures in the pulmonary arteries.

  • Cardiac catheterization to provide a definite diagnosis of pulmonary hypertension. A diagnosis of pulmonary hypertension is made if the pulmonary artery pressure is 25 mm Hg or greater while at rest.
  • Echocardiography to estimate pulmonary artery pressure. An estimated pulmonary artery pressure of 35 to 40 mm Hg or greater on echocardiography suggests pulmonary hypertension. A diagnosis of pulmonary hypertension may be made when enough changes are seen on an echocardiogram. If the echocardiogram suggests pulmonary hypertension, then right heart catheterization may be the next step.

Medical history and physical exam

Your doctor may ask you about any signs and symptoms you have been experiencing and any risk factors such as other medical conditions you have.

Your doctor will also perform a physical exam to look for signs that may help diagnose your condition. As part of this exam, your doctor may do the following:

  • Check whether the oxygen levels in your blood are low. This may be done by pulse oximetry, in which a probe is placed on your finger to check your oxygen levels.
  • Feel your liver to see if it is larger than normal.
  • Listen to your heart to see if there are changes in how it sounds, and also to find out if your heartbeat is faster than normal or irregular or if you have a new heart murmur.
  • Listen to your lungs for sounds that could be caused by heart failure or interstitial lung disease.
  • Look at the veins in your neck to see if they are larger than normal.
  • Look for swelling in your abdomen and legs that may be caused by fluid buildup.
  • Measure your blood pressure.

Diagnostic tests

Your doctor may order blood tests and imaging tests to help diagnose pulmonary hypertension.

  • Blood tests to look for increased risk of blood clots, stress on the heart, or anemia.
  • Cardiac MRI to get detailed pictures of the structure and functioning of the heart and surrounding blood vessels.
  • Chest X-ray to look at the size and shape of the heart and surrounding blood vessels, including the pulmonary arteries.
  • Echocardiogram (echo) to look for signs of pulmonary hypertension and also study the heart’s structure and functioning. If the echocardiogram suggests pulmonary hypertension, then right heart catheterization may be the next step.
  • Electrocardiogram (ECG or EKG) to look for signs of changes in your heart or abnormal rhythms in your heart’s electrical activity caused by pulmonary hypertension. You may still have pulmonary hypertension if you have a normal ECG.

Tests for other medical conditions

Your doctor may order additional tests to see whether another condition or medicine may be causing your pulmonary hypertension. Doctors can use this information to develop your treatment plan.

Reminders

  • Return to Risk Factors to review family history, medical conditions, or other factors that increase your risk of developing pulmonary hypertension.
  • Return to Signs, Symptoms, and Complications to review common signs and symptoms of pulmonary hypertension.

Treatment

If you are diagnosed with pulmonary hypertension, your doctor will determine your treatment plan based on the cause of disease, if it is known. Your doctor may recommend healthy lifestyle changes, medicines, or other treatments aimed at keeping your symptoms from getting worse, increasing your ability to exercise, improving heart function, and ensuring a better quality of life. There is no cure for pulmonary hypertension unless chronic blood clots in the lungs are the cause.

Healthy lifestyle changes

Depending on the cause of your pulmonary hypertension, your doctor may recommend healthy lifestyle changes.

  • Heart-healthy eating, which includes eating less salt, to lower blood pressure or cholesterol if high levels of these contributed to the cause of your pulmonary hypertension. Eating less salt will help control your body fluids and may improve heart function.
  • Physical activity as recommended and supervised by your doctor
  • Physical rehabilitation to improve your ability to exercise and also boost your quality of life

Medicines

Medicines to treat pulmonary hypertension may include:

  • Anticoagulation or blood thinners to prevent blood clots in people whose pulmonary hypertension is caused by chronic blood clots in the lungs. These thinners also can help some people who have pulmonary arterial hypertension, heart failure, or other risk factors for blood clots.
  • Digitalis, or digoxin to control the rate blood is pumped throughout the body.
  • Vasodilator therapy to relax blood vessels and lower blood pressure in the pulmonary artery most affected in people who have pulmonary arterial hypertension. This includes calcium channel blockers such as nifedipine and diltiazem, as well as newer groups of medicines called endothelin receptor antagonists and phosphodiesterase type 5 inhibitors.

Procedures and therapies

Your doctor may recommend a procedure, surgery, or therapy to treat pulmonary hypertension.

  • Oxygen therapy if oxygen levels in the blood are too low.
  • Balloon atrial septostomy to decrease pressure in the right heart chambers and improve the output of the left heart and oxygenation of the blood. In this procedure, a small hole is made in the wall between the right and left atria to allow blood to flow from the right to the left atrium.
  • Balloon pulmonary angioplasty to lower the blood pressure in your pulmonary artery and improve heart function in people who cannot have a pulmonary endarterectomy.
  • Pulmonary endarterectomy surgery to remove blood clots from the inside of the blood vessels of the lungs.

Treatments for other conditions

Your doctor may recommend medicines or procedures to treat the condition that is causing your pulmonary hypertension.

  • Blood pressure medicines such as angiotensin-converting enzymes inhibitors, beta blockers, or calcium channel blockers when left heart disease is the cause
  • Blood transfusions or hydroxyurea to treat sickle cell disease
  • Heart valve repair
  • Iron supplements to increase blood iron levels and improve anemia

Look for

  • Research for Your Health will discuss how we are using current research and advancing research to treat people with pulmonary hypertension.
  • Participate in NHLBI Clinical Trials will discuss our open and enrolling clinical studies that are investigating treatments for pulmonary hypertension.
  • Living With will discuss what your doctor may recommend, including lifelong lifestyle changes and medical care to prevent your condition from recurring, getting worse, or causing complications.

Living With

After you are diagnosed with pulmonary hypertension, it is important to follow your treatment plan, get regular care, and learn how to monitor your condition. Taking these steps can slow down the progression of the disease and may improve your condition. Your specific treatment plan will depend on the cause of your pulmonary hypertension, as well as how advanced it is.

Receive routine follow-up care

Your follow-up care may include recommendations such as these:

  • Participate in support groups, counseling, and education efforts that can help you manage the activities of daily living, experience a successful pregnancy, and generally improve the quality of your life.
  • Get the recommended vaccines, which often include a vaccine for pneumococcus and an influenza, or flu, shot every year at the start of flu season.

Return to Treatment to review possible treatment options for your pulmonary hypertension.

Monitor your condition

Talk to your doctor about new or concerning symptoms. People who have pulmonary hypertension may need regular tests. Your doctor may recommend the following to monitor your condition and treatment response:

  • Six-minute walk test to monitor your ability to exercise
  • Blood tests to check hemoglobin, iron, and electrolyte levels; kidney, liver, and thyroid function; your blood’s ability to clot; and signs of stress on the heart
  • Cardiac catheterization
  • Cardiac MRI to monitor your heart’s size and how well it is working
  • Chest X-ray
  • Echocardiography to monitor your heart’s size and how well it is working, and measure the pressure in your right heart chambers
  • Electrocardiogram to check for irregular heartbeats
  • Pulmonary function tests to check for any change in your lung function

If your pulmonary hypertension is severe or does not respond to treatment, your doctor may talk to you about a lung transplant or a heart and lung transplant.

Prevent complications over your lifetime

To help prevent some of the complications of pulmonary hypertension, your doctor may recommend the following.

  • Make heart-healthy lifestyle changes such as heart-healthy eating if your pulmonary hypertension is due to heart failure from ischemic heart disease or high blood pressure.
  • Engage in regular physical activity. Before starting any exercise program, ask your doctor about what level of physical activity is right for you.
  • Avoid high altitudes when possible and discuss with your doctor any plans for air travel or visits to places at high altitude.
  • Talk to your doctor if you are planning to get pregnant, as there is an increased risk of pregnancy complications.
  • Treat other medical conditions, such as COPD, heart conditions, and sleep apnea.

Learn the warning signs of serious complications and have a plan

Even with treatment, pulmonary hypertension may lead to serious complications such as heart failure and arrhythmias. Know the signs and symptoms of pulmonary hypertension and how to recognize the possible complications.

If you are taking a blood thinner, this will increase your risk of bleeding. If you experience any abnormal bleeding, such as blood in your stool, black stool, or coughing up blood, contact your doctor right away. If you fall while taking a blood thinner, you are at higher risk for bleeding inside your head. Let your doctor know if you have fallen while taking a blood thinner.

Some treatments for pulmonary hypertension must be given through a long-term intravenous (IV) line. Call your doctor right away if you have any signs of infection. Signs of infection include redness, swelling, or yellow discharge where the IV is inserted; a fever of 100.3°F or higher; and chills.

Research for Your Health

The NHLBI is part of the U.S. Department of Health and Human Services’ National Institutes of Health (NIH)—the Nation’s biomedical research agency that makes important scientific discovery to improve health and save lives. We are committed to advancing science and translating discoveries into clinical practice to promote the prevention and treatment of heart, lung, blood, and sleep disorders, including pulmonary hypertension. Learn about the current and future NHLBI efforts to improve health through research and scientific discovery.

Improving health with current research

Learn about the following ways the NHLBI continues to translate current research into improved health for people with pulmonary hypertension. Research on this topic is part of the NHLBI’s broader commitment to advancing lung disease scientific discovery.

  • The NHLBI Accelerates Translation of Discoveries into Innovative Approaches. The Vascular Interventions/Innovations and Therapeutic Advances (VITA) Program is a translational program that supports and accelerates early-stage development of promising diagnostics and treatments. VITA seeks to address unmet clinical needs for vascular diseases, particularly in underserved medical communities.
  • The NHLBI Advances Personalized Medicine for Pulmonary Hypertension. The NHLBI and the Cardiovascular Medical Research and Education Fund held a workshop that addressed multiple topics that are central to the development of true personalized medicine in pulmonary vascular disease, including pulmonary hypertension. The workshop also identified needs and made recommendations to the NHLBI for future research priorities in this area.
  • The NHLBI Stimulates New Treatment Approaches for Pulmonary Hypertension. Our Centers for Advanced Diagnostics and Experimental Therapeutics in Lung Diseases (CADET) program was created to fund the development of new drugs and diagnostics for pulmonary diseases and sleep-disordered breathing. Researchers in this program have discovered a new way to stop and potentially reverse the changes that occur with pulmonary hypertension through the use of antibodies that target two proteins which contribute to the disease. Visit NHLBI Supports CADET Researchers to Produce New Pulmonary Disease Drugs (Part 3) for more information.

Learn about the pioneering research contributions we have made that improved clinical care.

Advancing research for improved health

In support of our mission, we are committed to advancing pulmonary hypertension research in part through the following ways.

  • We perform research. Our Division of Intramural Research, which includes investigators from the Sickle Cell Branch, is actively engaged in the study of pulmonary hypertension.
  • We fund research. The research we fund today will help improve our future health. Our Division of Lung Diseases and its Lung Biology and Disease Branch oversee much of the research on pulmonary hypertension that we fund. Search the NIH RePORTER to learn about research the NHLBI funds on pulmonary hypertension.
  • We stimulate high-impact research. Our Trans-Omics for Precision Medicine (TOPMed) Program now includes participants who have pulmonary hypertension. This work may help us understand how genes contribute to differences in disease severity and to how patients respond to treatment, particularly in patients who have sickle cell disease. The NHLBI Strategic Vision highlights ways we may support research over the next decade, including research on pulmonary hypertension.

Learn about exciting research areas the NHLBI is exploring about pulmonary hypertension.

Participate in NHLBI Clinical Trials

We lead or sponsor many studies on pulmonary hypertension. See whether you or someone you know is eligible to participate in our clinical trials.

Are you a healthy adult, or do you have pulmonary hypertension and normal pump function of your heart?

This study is exploring how a medicine called an oral nitrate gets broken down in healthy adults, compared with how it breaks down in those who have pulmonary hypertension with normal pump function of their heart, also known as heart failure with preserved ejection fraction (PH-HFpEF). Researchers will study the microbiome of various patient biological samples. To participate in this study, you must be at least 18 years old and either be healthy or have PH-HFpEF that was confirmed by right heart catheterization in the past 6 months. This study is located in Pittsburgh, Pennsylvania.

Are you an adult with obesity and pulmonary hypertension who is interested in a new treatment?

This study is looking at a medicine called metformin, which is normally used to treat diabetes, as possible treatment for pulmonary hypertension in people who have obesity. To participate in this study, you must be 30 to 75 years old, have a body mass index (BMI) of 30 or greater, and have experienced shortness of breath. This study is located in Boston, Massachusetts.

Do you or a family member have pulmonary hypertension?

This study is looking at how hormones contribute to pulmonary arterial hypertension and whether treatments for hormone imbalances or insulin resistance also improve pulmonary hypertension. To participate in this study, you must be 90 years old or younger and have pulmonary arterial hypertension or be a family member of someone who has this condition. This study is located in Nashville, Tennessee.

Are you an adult with pulmonary hypertension and normal pump function of your heart?

This study is looking at the effect of an oral medicine called a nitrate on the ability to exercise in people who have pulmonary hypertension and normal pump function of their heart, also known as pulmonary hypertension and heart failure with preserved ejection fraction (PH-HFpEF). To participate in this study, you must be at least 18 years old and have PH-HFpEF that was confirmed by right heart catheterization. This study is located in Pittsburgh, Pennsylvania.

Do you or your child have pulmonary hypertension and want to help research?

This study is looking to better understand pulmonary hypertension in children who have this condition. To participate in this study, you or your child must be 21 years old or younger and must have been diagnosed with pulmonary hypertension before the age of 18. This study is located at multiple sites across the United States and Canada, including California, Colorado, Massachusetts, New York, Pennsylvania, Tennessee, and Alberta, Canada.
This study aims to better understand the patterns of unstable breathing and heart blood flow, including pulmonary hypertension, that are seen in premature infants. To participate in this study, your infant must have been born prematurely with a gestational age between 24 weeks and 28 weeks and 6 days, and be a patient in the neonatal intensive care unit at St. Louis Children’s Hospital. This study is located in St. Louis, Missouri.

Are you an adult with pulmonary hypertension who would like to see whether a healthy eating plan and exercise can help?

This study is investigating the effects of diet and exercise on pulmonary arterial hypertension. Researchers want to know whether the way sugar is metabolized may contribute to pulmonary hypertension. This study also wants to find out whether healthy eating plans and physical activity can improve pulmonary hypertension. To participate in this study, you must be between 18 and 75 years old and have been diagnosed with pulmonary arterial hypertension. This study is located in Cleveland, Ohio.

Are you an adult interested in a new medicine to treat pulmonary hypertension?

This study is looking at whether a medicine called anastrozole, currently used to treat breast cancer, improves exercise capacity in patients who have pulmonary hypertension. To participate in this study, you must be at least 18 years old, have pulmonary arterial hypertension, and be able to perform a 6-minute walk test. This study is located at multiple sites across the United States, including California, Colorado, Maryland, Missouri, Pennsylvania, Rhode Island, and Tennessee.

Are you an adult interested in new technologies to study pulmonary hypertension?

This study is looking at new technologies that make it possible to study similarities and differences in genetic, molecular, and cellular processes of many diseases that are associated with pulmonary hypertension. To participate in this study, you must be at least 18 years old, be referred for right heart catheterization, and have or be at risk for pulmonary hypertension. This study is located at multiple sites across the United States, including Arizona, Maryland, Massachusetts, Minnesota, New York, and Tennessee.

Are you or your child going to have heart catheterization?

This study is investigating new techniques to perform heart catheterization using magnetic resonance imaging (MRI). Heart catheterization procedures are currently guided using X-rays, which exposes the patient to small amounts of radiation. This new MRI technique would allow the procedure to be performed without X-ray radiation. To participate in this study, you or your child must be at least 2 years old, scheduled to have heart catheterization, and able to undergo MRI. This study is located in Bethesda, Maryland.

Has your doctor recommended right heart catheterization for you?

This study is investigating new magnetic resonance imaging (MRI) techniques that use a guidewire to help position a heart catheter within the heart. MRI fluoroscopy shows pictures of the heart so that doctors can watch while they work. Using the guidewire during MRI may improve the procedure of heart catheterization. To participate in this study, you must be 18 to 99 years old, and your doctor must have recommended right heart catheterization for you. This study is located in Bethesda, Maryland.

Are you an adult interested in a new treatment for pulmonary hypertension?

This study is exploring whether a medicine called spironolactone can improve heart and lung function during exercise in people who have pulmonary hypertension. To participate in this study, you must be 18 to 99 years old and have a type of pulmonary hypertension known as pulmonary arterial hypertension. This study is located in Bethesda, Maryland.

Do you know a child or young adult recently treated for VTE?

This study is exploring why some people experience complications after treatment for venous thromboembolism (VTE), which includes deep vein thrombosis and pulmonary embolism. To participate in this study, you must have been recently treated for VTE and be younger than 21 years old. This study is located in Dallas, Texas.
More Information

After reading our Pulmonary Hypertension Health Topic, you may be interested in additional information found in the following resources.

Non-NHLBI resources

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