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.
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.
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:
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.
You may be at an increased risk of pulmonary hypertension if you have or are exposed to the following:
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.
Unhealthy lifestyle habits can increase the risk of pulmonary hypertension. These habits include:
Some medicines may increase your risk of pulmonary hypertension, including:
Certain medical conditions may increase your risk of developing pulmonary hypertension:
Pulmonary hypertension is more common in women than in men. Pulmonary hypertension with certain types of heart failure is also more common in women.
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.
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.
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.
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.
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 of pulmonary hypertension may include the following:
Complications of pulmonary hypertension may include the following:
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.
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.
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:
Your doctor may order blood tests and imaging tests to help diagnose pulmonary hypertension.
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.
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.
Depending on the cause of your pulmonary hypertension, your doctor may recommend healthy lifestyle changes.
Medicines to treat pulmonary hypertension may include:
Your doctor may recommend a procedure, surgery, or therapy to treat pulmonary hypertension.
Your doctor may recommend medicines or procedures to treat the condition that is causing your pulmonary hypertension.
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.
Your follow-up care may include recommendations such as these:
Return to Treatment to review possible treatment options for your pulmonary hypertension.
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:
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.
To help prevent some of the complications of pulmonary hypertension, your doctor may recommend the following.
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.
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.
Learn about the pioneering research contributions we have made that improved clinical care.
In support of our mission, we are committed to advancing pulmonary hypertension research in part through the following ways.
Learn about exciting research areas the NHLBI is exploring about pulmonary hypertension.
We lead or sponsor many studies on pulmonary hypertension. See whether you or someone you know is eligible to participate in our clinical trials.
After reading our Pulmonary Hypertension Health Topic, you may be interested in additional information found in the following resources.