Pulmonary hypertension is a type of high blood pressure that affects the arteries in the lungs and the right side of the heart.
In one form of pulmonary hypertension, called pulmonary arterial hypertension (PAH), blood vessels in the lungs are narrowed, blocked or destroyed. The damage slows blood flow through the lungs. Blood pressure in the lung arteries goes up. The heart must work harder to pump blood through the lungs. The extra effort eventually causes the heart muscle to become weak and fail.
In some people, pulmonary hypertension slowly gets worse and can be life-threatening. There's no cure for pulmonary hypertension. But treatments are available to help you feel better, live longer and improve your quality of life.
The 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, at first while exercising and eventually while at rest.
Blue or gray skin color due to low oxygen levels. Depending on your skin color, these changes may be harder or easier to see.
Chest pressure or pain.
Dizziness or fainting spells.
Fast pulse or pounding heartbeat.
Fatigue.
Swelling in the ankles, legs and belly area.
Shortness of breath is the most common symptom of pulmonary hypertension. But it may be caused by other health conditions such as asthma. See a health care professional for an accurate diagnosis.
There's no cure for pulmonary hypertension. But treatment is available to improve symptoms and prolong life, and to keep the disease from getting worse. You also may get treatments for any health problem that might be causing pulmonary hypertension.
It often takes some time to find the most appropriate treatment for pulmonary hypertension. The treatments are often complex. You usually need a lot of health checkups.
Medications
If you have pulmonary hypertension, you may get medicines to treat your symptoms and help you feel better. Medicines also may be used to treat or prevent complications. Treatment may include:
Medicines to relax blood vessels. Also called vasodilators, these medicines help open narrowed blood vessels and improve blood flow. The medicine comes in many forms. It may be breathed in, taken by mouth or given by IV. Some types are given continuously through a small pump attached to the body.
Examples of vasodilators to treat pulmonary hypertension include epoprostenol (Flolan, Veletr), treprostinil (Remodulin, Tyvaso, others), Iloprost (Ventavis) and selexipag (Uptravi).
Soluble guanylate cyclase (sGC) stimulators. This type of medicine relaxes the pulmonary arteries and lowers pressure in the lungs. Examples include riociguat (Adempas). Do not take these medicines if you're pregnant.
Medicines to widen blood vessels. Medicines called endothelin receptor antagonists reverse the effect of a substance in the walls of blood vessels that causes them to narrow. Such medicines include bosentan (Tracleer), macitentan (Opsumit) and ambrisentan (Letairis). They may improve energy level and symptoms. Do not take these medicines if you are pregnant.
Medicines to increase blood flow. Medicines called phosphodiesterase 5 (PDE5) inhibitors may be used to increase blood flow through the lungs. These medicines also are used to treat erectile dysfunction. They include sildenafil (Revatio, Viagra) and tadalafil (Adcirca, Alyq, Cialis).
High-dose calcium channel blockers. These medicines help relax the muscles in the walls of blood vessels. They include amlodipine (Norvasc), diltiazem (Cardizem, Tiazac, others) and nifedipine (Procardia). Although calcium channel blockers can be effective, only a small number of people with pulmonary hypertension improve while taking them.
Blood thinners. Also called anticoagulants, these medicines help prevent blood clots. One example is warfarin (Jantoven). Blood-thinning medicines slow the clotting process. The medicines can increase the risk of bleeding. This is especially true if you're having surgery or a procedure that enters the body or creates an opening in the skin. Talk to your health care team about your risk.
Digoxin (Lanoxin). This medicine helps the heart beat stronger and pump more blood. It can help control irregular heartbeats.
Water pills, also called diuretics. These medicines help the kidneys remove excess fluid from the body. This reduces the amount of work the heart has to do. Diuretics also may be used to reduce fluid buildup in the lungs, legs and belly area.
Oxygen therapy. Breathing pure oxygen is sometimes recommended as a treatment for pulmonary hypertension. This treatment may be suggested if you live at a high altitude or have sleep apnea. Some people with pulmonary hypertension need oxygen therapy all the time.
Surgery or other procedures
If medicines do not help control the symptoms of pulmonary hypertension, surgery may be recommended. Surgeries and procedures to treat pulmonary hypertension may include:
Atrial septostomy. This treatment may be recommended if medicines don't control pulmonary hypertension symptoms. In an atrial septostomy, a doctor creates an opening between the upper left and right chambers of the heart. The opening reduces the pressure on the right side of the heart. Potential complications include irregular heartbeats called arrhythmias.
Lung or heart-lung transplant. Sometimes, a lung or heart-lung transplant may be needed, especially for younger people who have idiopathic pulmonary arterial hypertension. After a transplant, medicine must be taken for life to help reduce the chance of rejection.
Diagnosis
Pulmonary hypertension is hard to diagnose early because 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 health care professional examines you and asks about your symptoms. You'll likely be asked questions about your medical and family history.
Tests
Tests done to help diagnose pulmonary hypertension may include:
Blood tests. Blood tests can help find the cause of pulmonary hypertension or show signs of complications.
Chest X-ray. A chest X-ray creates pictures 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 can show changes in the heartbeat.
Echocardiogram. Sound waves are used to create moving images of the beating heart. An echocardiogram shows blood flow through the heart. This test may be done to help diagnose pulmonary hypertension or to determine how well 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 cardiologist places a thin, flexible tube called a catheter into a blood vessel, usually in the neck. The catheter is gently guided into the lower right heart chamber and the pulmonary artery. A 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 create cross-sectional images 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 such as COPD or pulmonary fibrosis.
Magnetic resonance imaging (MRI). This test uses magnetic fields and radio waves to create detailed images of the heart. It can show blood flow in the pulmonary arteries and determine 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 blood flow. You also may breathe in a tracer that shows airflow to the lungs. A V/Q scan can show 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 health care team may use a risk calculator that considers your symptoms and test results to understand what type of treatment is needed. This is called pulmonary hypertension risk stratification.
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