Pulmonary hypertension

Overview

Pulmonary hypertension is a type of high blood pressure that affects the arteries in your lungs and the right side of your heart.

In one form of pulmonary hypertension, called pulmonary arterial hypertension (PAH), blood vessels in your lungs are narrowed, blocked or destroyed. The damage slows blood flow through your lungs, and blood pressure in the lung arteries rises. Your heart must work harder to pump blood through your lungs. The extra effort eventually causes your heart muscle to become weak and fail.

In some people, pulmonary hypertension slowly gets worse and can be life-threatening. Although there's no cure for some types of pulmonary hypertension, treatment can help reduce symptoms and improve your quality of life.

Blood flow in lungs, and normal and blocked pulmonary arteries

When the tiny blood vessels in your lungs become thickened, narrowed, blocked or destroyed, it's harder for blood to flow through the lungs. As a result, blood pressure increases in the lungs, a condition called pulmonary hypertension.

Symptoms

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

Pulmonary hypertension symptoms include:

  • Shortness of breath (dyspnea), initially while exercising and eventually while at rest
  • Fatigue
  • Dizziness or fainting spells (syncope)
  • Chest pressure or pain
  • Swelling (edema) in your ankles, legs and eventually in your abdomen (ascites)
  • Bluish color to your lips and skin (cyanosis)
  • Racing pulse or heart palpitations

Causes

Your heart has two upper chambers (atria) and two lower chambers (ventricles). Each time blood passes through your heart, the lower right chamber (right ventricle) pumps blood to your lungs through a large blood vessel (pulmonary artery).

In your lungs, the blood releases carbon dioxide and picks up oxygen. The blood normally flows easily through blood vessels in your lungs (pulmonary arteries, capillaries and veins) to the left side of your heart.

However, changes in the cells that line your pulmonary arteries can cause the walls of the arteries to become stiff, swollen and thick. These changes may slow down or block blood flow through the lungs, causing pulmonary hypertension.

Pulmonary hypertension is classified into five groups, depending on the cause.

Group 1: Pulmonary arterial hypertension (PAH)

Causes include:

  • Unknown cause (idiopathic pulmonary arterial hypertension)
  • A genetic mutation passed down through families (heritable pulmonary arterial hypertension)
  • Use of some prescription diet drugs or illegal drugs such as methamphetamines — and other drugs
  • Heart problems present at birth (congenital heart disease)
  • Other conditions, such as connective tissue disorders (scleroderma, lupus, others), HIV infection or chronic liver disease (cirrhosis)

Group 2: Pulmonary hypertension caused by left-sided heart disease

Causes include:

  • Left-sided heart valve disease, such as mitral valve or aortic valve disease
  • Failure of the lower left heart chamber (left ventricle)

Group 3: Pulmonary hypertension caused by lung disease

Causes include:

  • Chronic obstructive pulmonary disease (COPD)
  • Pulmonary fibrosis, a condition that causes scarring in the tissue between the lungs' air sacs (interstitium)
  • Obstructive sleep apnea
  • Long-term exposure to high altitudes in people who may be at higher risk of pulmonary hypertension

Group 4: Pulmonary hypertension caused by chronic blood clots

Causes include:

  • Chronic blood clots in the lungs (pulmonary emboli)
  • Other clotting disorders

Group 5: Pulmonary hypertension triggered by other health conditions

Causes include:

  • Blood disorders, including polycythemia vera and essential thrombocythemia
  • Inflammatory disorders such as sarcoidosis and vasculitis
  • Metabolic disorders, including glycogen storage disease
  • Kidney disease
  • Tumors pressing against pulmonary arteries

Eisenmenger syndrome and pulmonary hypertension

Eisenmenger syndrome is a type of congenital heart disease that causes pulmonary hypertension. It's most commonly caused by a large hole in your heart between the two lower heart chambers (ventricles), called a ventricular septal defect.

This hole in your heart causes blood to flow incorrectly in your heart. Oxygen-carrying blood (red blood) mixes with oxygen-poor blood (blue blood). The blood then returns to your lungs — instead of going to the rest of your body — increasing the pressure in the pulmonary arteries and causing pulmonary hypertension.

Chambers and valves of the heart

A normal heart has two upper and two lower chambers. The upper chambers, the right and left atria, receive incoming blood. The lower chambers, the more muscular right and left ventricles, pump blood out of your heart. The heart valves, which keep blood flowing in the right direction, are gates at the chamber openings.

Risk factors

Growing older can increase your risk of developing pulmonary hypertension. The condition is more often diagnosed in people ages 30 to 60. However, idiopathic PAH is more common in younger adults.

Other things that can raise your risk of pulmonary hypertension include:

  • A family history of the condition
  • Being overweight
  • Blood-clotting disorders or a family history of blood clots in the lungs
  • Exposure to asbestos
  • Genetic disorders, including congenital heart disease
  • Living at a high altitude
  • Use of certain weight-loss drugs
  • Use of illegal drugs such as cocaine
  • Use of selective serotonin reuptake inhibitors (SSRIs), used to treat depression and anxiety

Complications

Complications of pulmonary hypertension include:

  • Right-sided heart enlargement and heart failure (cor pulmonale). In cor pulmonale, your heart's right ventricle becomes enlarged and has to pump harder than usual to move blood through narrowed or blocked pulmonary arteries.

    At first, the heart tries to compensate by thickening its walls and expanding the chamber of the right ventricle to increase the amount of blood it can hold. But these changes create more strain on the heart, and eventually the right ventricle fails.

  • Blood clots. Having pulmonary hypertension makes it more likely you'll develop clots in the small arteries in your lungs, which is dangerous if you already have narrowed or blocked blood vessels.
  • Arrhythmia. Pulmonary hypertension can cause irregular heartbeats (arrhythmias), which can lead to a pounding heartbeat (palpitations), dizziness or fainting. Certain arrhythmias can be life-threatening.
  • Bleeding in the lungs. Pulmonary hypertension can lead to life-threatening bleeding into the lungs and coughing up blood (hemoptysis).
  • Pregnancy complications. Pulmonary hypertension can be life-threatening for a woman and her developing baby.

Diagnosis

Pulmonary hypertension is hard to diagnose early because it's not often detected in a routine physical exam. Even when the condition is more advanced, its signs and symptoms are similar to those of other heart and lung conditions.

Your doctor will perform a physical exam and talk to you about your signs and symptoms. You'll be asked questions about your medical and family history.

Your doctor will order tests to help diagnose pulmonary hypertension and determine its cause. Tests for pulmonary hypertension may include:

  • Blood tests. Blood tests can help your doctor determine the cause of pulmonary hypertension or look for signs of complications.
  • Chest X-ray. A chest X-ray creates pictures of your heart, lungs and chest. It can show enlargement of the right ventricle of the heart or the pulmonary arteries, which can occur in pulmonary hypertension. Your doctor may also use a chest X-ray to check for other lung conditions that can cause pulmonary hypertension.
  • Electrocardiogram (ECG). This noninvasive test shows your heart's electrical patterns and can detect abnormal heartbeats. An ECG may also reveal signs of right ventricle enlargement or strain.
  • Echocardiogram. Sound waves can create moving images of the beating heart. An echocardiogram lets your doctor see how well your heart and its valves are working. It can be used to determine the size and thickness of the right ventricle, and to measure the pressure in your pulmonary arteries.

    Sometimes, an echocardiogram is done while you exercise on a stationary bike or treadmill to understand how well your heart works during activity. You may be asked to wear a mask that checks how well your heart and lungs use oxygen and carbon dioxide.

    An echocardiogram may also be done after diagnosis to assess how your treatments are working.

  • Right heart catheterization. If an echocardiogram reveals pulmonary hypertension, you'll likely have a right heart catheterization to confirm the diagnosis.

    During this procedure, a cardiologist places a thin, flexible tube (catheter) into a vein in your neck or groin. The catheter is then threaded into your right ventricle and pulmonary artery.

    Right heart catheterization allows your doctor to directly measure the pressure in the main pulmonary arteries and right ventricle of the heart. It's also used to see what effect different medications may have on your pulmonary hypertension.

Your doctor might also order one or more of the following tests to check the condition of your lungs and pulmonary arteries and further determine the cause of pulmonary hypertension:

  • Computerized tomography (CT). This imaging test creates cross-sectional pictures of the bones, blood vessels and soft tissues inside your body. A CT scan can show the heart's size, spot blood clots in the lungs' arteries, and look closely for lung diseases that might lead to pulmonary hypertension, such as COPD or pulmonary fibrosis.

    Sometimes, a special dye, called contrast, is injected into your blood vessels before the CT scan (CT angiography). The dye helps your arteries show up more clearly on the images.

  • Magnetic resonance imaging (MRI). An MRI scan uses a magnetic field and pulses of radio wave energy to make pictures of the body. Your doctor may order this test to check the right ventricle's function and blood flow in the lung's arteries.
  • Pulmonary function test. This noninvasive test measures how much air your lungs can hold, and the airflow in and out of your lungs. During the test, you'll blow into a simple instrument called a spirometer.
  • Polysomnogram. This test measures your brain activity, heart rate, blood pressure, oxygen levels and other factors while you sleep. It can help diagnose a sleep disorder such as obstructive sleep apnea, which can cause pulmonary hypertension.
  • Ventilation/perfusion (V/Q). In this test, a tracer is injected into a vein in your arm. The tracer shows blood flow and air to your lungs. A V/Q test can determine whether blood clots are causing symptoms of pulmonary hypertension.
  • Open-lung biopsy. Rarely, a doctor might recommend an open-lung biopsy to check for a possible cause of pulmonary hypertension. An open-lung biopsy is a type of surgery in which a small sample of tissue is removed from your lungs while you are under general anesthesia.

Genetic tests

If a family member has had pulmonary hypertension, your doctor might screen you for genes that are linked with pulmonary hypertension. If you test positive, your doctor might recommend that other family members also be screened.

Pulmonary hypertension classifications

Once you've been diagnosed with pulmonary hypertension, your doctor might classify the severity of your disease into one of several classes, including:

  • Class I. Although you've been diagnosed with pulmonary hypertension, you have no symptoms with normal activity.
  • Class II. You don't have symptoms at rest, but you have symptoms such as fatigue, shortness of breath or chest pain with normal activity.
  • Class III. You're comfortable at rest, but have symptoms when you're physically active.
  • Class IV. You have symptoms while at rest and during physical activity.

Treatment

There's no cure for pulmonary hypertension, but your doctors can prescribe treatments to help you manage your condition. Treatment may help improve your symptoms and slow the progress of pulmonary hypertension.

It often takes some time to find the most appropriate treatment for pulmonary hypertension. The treatments are often complex and require extensive follow-up care.

When pulmonary hypertension is caused by another condition, your doctor will treat the underlying cause whenever possible.

Medications

  • Blood vessel dilators (vasodilators). Vasodilators relax and open narrowed blood vessels, improving blood flow. One of the most commonly prescribed vasodilators for pulmonary hypertension is epoprostenol (Flolan, Veletri).

    This drug continuously flows through an intravenous (IV) line attached to a small pump, which you wear in a pack on your belt or shoulder. Potential side effects of epoprostenol include jaw pain, nausea, diarrhea, leg cramps, and pain and infection at the IV site.

    Other types of vasodilators, including treprostinil (Tyvaso, Remodulin, Orenitram), can be inhaled, injected, or taken by mouth. The drug iloprost (Ventavis) is given while you breathe in through a nebulizer, a machine that vaporizes your medication.

    Side effects associated with treprostinil include chest pain, often with headache and nausea, and breathlessness. Possible side effects of iloprost include headache, nausea and diarrhea.

  • Guanylate cyclase (GSC) stimulators. Riociguat (Adempas) increases nitric oxide in the body, which relaxes the pulmonary arteries and lowers pressure within them. Side effects include nausea, dizziness and fainting. You should not take GSC stimulators if you're pregnant.
  • Endothelin receptor antagonists. These medications reverse the effect of endothelin, a substance in the walls of blood vessels that causes them to narrow. Such drugs include bosentan (Tracleer), macitentan (Opsumit), and ambrisentan (Letairis). These drugs may improve your energy level and symptoms. However, they can damage your liver. You may need monthly blood tests to check your liver function. Endothelin receptor antagonists shouldn't be taken if you're pregnant.
  • Sildenafil and tadalafil. Sildenafil (Revatio, Viagra) and tadalafil (Adcirca, Cialis) are commonly used to treat erectile dysfunction. But they also open the blood vessels in the lungs and allow blood to flow through more easily. Side effects can include an upset stomach, headache and vision problems.
  • High-dose calcium channel blockers. These drugs help relax the muscles in the walls of your blood vessels. They include amlodipine (Norvasc), diltiazem (Cardizem, Tiazac, others) and nifedipine (Procardia, others). Although calcium channel blockers can be effective, only a small number of people with pulmonary hypertension improve while taking them.
  • Warfarin. Warfarin is a type of drug called an anticoagulant (blood thinner). Your doctor is likely to prescribe warfarin (Coumadin, Jantoven) to help prevent blood clots in the lung's arteries. This medication delays the clotting process and might put you at risk of bleeding, especially if you're having surgery or an invasive procedure. Talk to your doctor about whether you need to stop taking a blood-thinning medication before surgery and for how long.

    Many other drugs, herbal supplements and foods can interact with warfarin, so talk to your doctor about your diet and other medications. You'll need occasional blood tests while taking warfarin to check how well it's working.

  • Digoxin. Digoxin (Lanoxin) helps the heartbeat stronger and pump more blood. It can help control the heart rate if you have arrhythmias.
  • Diuretics. Commonly known as water pills, these medications help your kidneys remove excess fluid from the body. This reduces the amount of work your heart has to do. They may also be used to limit fluid buildup in your lungs, legs and abdomen.
  • Oxygen therapy. Your doctor might suggest that you sometimes breathe pure oxygen to help treat pulmonary hypertension, especially if you live at a high altitude or have sleep apnea. Some people who have pulmonary hypertension eventually need continuous oxygen therapy.

Surgery

  • Atrial septostomy. If medications don't control your pulmonary hypertension, this open-heart surgery might be an option. In an atrial septostomy, a surgeon creates an opening between the upper left and right chambers of your heart (atria) to relieve the pressure on the right side of your heart.

    Atrial septostomy can have serious complications, including heart rhythm problems (arrhythmias).

  • Transplantation. In some cases, a lung or heart-lung transplant might be an option, especially for younger people who have idiopathic pulmonary arterial hypertension.

    Major risks of any type of transplantation include rejection of the transplanted organ and serious infection. You must take immunosuppressant drugs for life to help reduce the chance of rejection.

Lifestyle and home remedies

Although medical treatment can't cure pulmonary hypertension, it can lessen symptoms. Lifestyle changes also can help improve your condition. Consider these tips:

  • Get plenty of rest. Resting can reduce the fatigue that might come from having pulmonary hypertension.
  • Stay as active as possible. Even the mildest forms of activity might be too exhausting for some people who have pulmonary hypertension. For others, moderate exercise such as walking might be beneficial — especially when done during oxygen therapy. But first, talk to your doctor about specific exercise restrictions.

    In most cases, it's recommended that people with pulmonary hypertension not lift heavy weights. Your doctor can help you plan an appropriate exercise program.

  • Don't smoke. If you smoke, the most important thing you can do for your heart and lungs is to stop. If you can't stop smoking by yourself, ask your doctor to prescribe a treatment plan to help you quit. Also, avoid secondhand smoke if possible.
  • Avoid pregnancy and birth control pills. Birth control pills can increase the risk of blood clots. Talk to your doctor about alternative forms of birth control. If you do become pregnant, it's important to consult with your doctor as pulmonary hypertension can cause serious complications to both mom and her baby.
  • Don't travel to or live at high altitudes. High altitudes can worsen the symptoms of pulmonary hypertension. If you live at an altitude of 8,000 feet (2,438 meters) or higher, your doctor might recommend that you move to a lower altitude.
  • Avoid situations that can excessively lower blood pressure. These include sitting in a hot tub or sauna or taking long hot baths or showers. These activities lower your blood pressure and can cause fainting or even death. Also avoid activities that cause prolonged straining, such as lifting heavy objects or weights.
  • Eat healthy and manage your weight. Aim to eat a healthy diet rich in whole grains, fruits and vegetables, lean meats and low-fat dairy products. Avoid saturated fat, trans fat and cholesterol. It's likely your doctor will recommend limiting the amount of salt in your diet. Aim to maintain a healthy weight.
  • Ask your doctor about medications. Take all your medications as prescribed. Ask your doctor about any other medications before taking them, as some can interfere with pulmonary hypertension treatments or make your symptoms worse.
  • Get regular doctor checkups. Your doctor may recommend regular follow-up appointments. Let your doctor know if you have any questions about your condition or medications you're taking, or if you have any symptoms or side effects from your medications. If pulmonary hypertension affects your quality of life, ask your doctor about options that could help. Your doctor may work together with another specialist to initiate and manage any medications for pulmonary hypertension.
  • Get recommended vaccines. Your doctor may recommend getting an influenza and pneumonia vaccine, as these conditions can cause serious issues for people with pulmonary hypertension.
  • Get support. If you're feeling stressed or worried due to your condition, get support from family or friends. Or, consider joining a support group with others who have pulmonary hypertension.

Preparing for an appointment

If you think you might have pulmonary hypertension or are worried about your pulmonary hypertension risk, make an appointment with your family doctor.

While shortness of breath is one of the first symptoms of pulmonary hypertension, the symptom is also common with many other conditions, such as asthma.

Because appointments can be brief, and because there's often a lot to discuss, it's a good idea to be prepared for your appointment. Here's some information to help you get ready for your appointment, and what to expect from your doctor.

What you can do

  • Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as fill out forms or restrict your diet. For some imaging tests, for example, you might need to fast for a period of time beforehand.
  • Write down any symptoms you're experiencing, including any that might seem unrelated to pulmonary hypertension. Try to recall when they began. Be specific, such as days, weeks, months, and avoid vague terms such as "some time ago."
  • Write down key personal information, including a family history of pulmonary hypertension, lung disease, heart disease, stroke, high blood pressure or diabetes, and any major stresses or recent life changes.
  • Make a list of all medications, as well as any vitamins or supplements that you're taking. Also, be sure to tell your doctor if you've recently stopped taking any medications.
  • Take a family member or friend along, if possible. Sometimes it can be difficult to remember all the information provided to you during an appointment. Someone who accompanies you might remember something that you missed or forgot.
  • Be prepared to discuss your diet and exercise habits. If you don't already follow a diet or exercise routine, be ready to talk to your doctor about any challenges you might face in getting started.
  • Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions will help you make the most of your time together. List your questions from most important to least important in case time runs out. For pulmonary hypertension, some basic questions to ask your doctor include:

  • What is likely causing my symptoms or condition?
  • What are other possible causes for my symptoms or condition?
  • What kinds of tests will I need?
  • What's the most appropriate treatment?
  • What's an appropriate level of physical activity?
  • How often should I be screened for changes in my condition?
  • What are the alternatives to the primary approach that you're suggesting?
  • I have other health conditions. How can I best manage them together?
  • Are there any restrictions that I need to follow?
  • Should I see a specialist?
  • Is there a generic alternative to the medicine you're prescribing?
  • Are there any brochures or other printed material that I can take home with me? What websites do you recommend?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask other questions during your appointment.

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them might reserve time to go over any points you want to spend more time on. Your doctor might ask:

  • When did you first begin experiencing symptoms?
  • Have your symptoms been continuous or occasional?
  • How severe are your symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, seems to worsen your symptoms?

What you can do in the meantime

It's never too early to make healthy lifestyle changes, such as quitting smoking, cutting down on salt and eating a healthy diet. These changes can help prevent pulmonary hypertension from getting worse.

Last Updated Mar 20, 2020


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