Pulmonary hypertension is hard to diagnose because it may not show up on a routine physical exam and symptoms can be similar to many lung and heart problems. But it is important to recognize the condition and get treatment.
Your health care team may order a variety of tests. They include an echocardiogram, which uses sound waves to create moving pictures of your heart.
Symptoms
Feeling light-headed and dizzy when you exercise is one of the early symptoms of pulmonary hypertension (PH). It happens because blood vessels in your heart and lungs become narrow, which makes it harder for your body to get the oxygen it needs. You may also notice that your breath becomes shallow or faster when you exercise. Your health care professional can diagnose pulmonary hypertension through a physical exam, which includes feeling your abdomen and sternum. He or she will also listen to your lungs with a stethoscope and look at your belly, ankles and legs for signs of edema. If your health care professional thinks you have pulmonary hypertension, he or she will order tests to check for the condition and see how well your heart and lungs work.
If your PH is caused by blood clots, you’ll have an echocardiogram to find them. You’ll also have a ventilation-perfusion scan to see whether your lungs are being overworked, which can be a sign of the disease. Your doctor will also order blood tests to check your general health, the function of your organs and your hormone levels. Chest X-rays, an electrocardiogram and right-heart catheterization may also be needed to help make a diagnosis.
Pulmonary hypertension can be hard to diagnose because the symptoms are similar to those of many other diseases. Sometimes the cause of pulmonary hypertension isn’t known, which is called idiopathic pulmonary hypertension. Other times, the disease is a result of other conditions such as chronic bronchitis or emphysema.
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Once a diagnosis has been made, you and your doctor can plan treatment. Medicines can ease some of the symptoms and lower your blood pressure in the lungs. These drugs include vasodilators, which widen blood vessels and make it easier for your heart to pump. You can also take medications that reduce the risk of blood clots, such as anticoagulants, and diuretics, which help your body get rid of excess fluid. These medicines may be taken orally, by injection or through a nebulizer. You may also need to use supplemental oxygen at home or in the hospital.
Diagnosis
Pulmonary hypertension is usually diagnosed based on your medical history, symptoms and results of certain tests. Your healthcare team may also check your family history for gene changes that can cause pulmonary hypertension.
You’ll have a physical exam and blood tests. Your healthcare provider will ask about any symptoms you have and when they started. They’ll want to know if the symptoms get worse with exercise or are a constant part of your life. They’ll also ask about other medical conditions you have, such as lung disease or kidney disease.
Your provider will order an echocardiogram. This test uses sound waves to create moving images of your heart. It can show whether your heart’s right ventricle or pulmonary arteries are larger than usual. A Doppler echocardiogram can also measure blood flow through your heart valves and show how much oxygen your lungs are getting. Your provider might also order a chest CT scan to look for other heart or lung problems. A polysomnogram, or overnight sleep test, can help find if you have a sleep disorder like sleep apnea that could be making your pulmonary hypertension worse. Your provider might also do a ventilation/perfusion scan to see how much oxygen is being delivered to your lungs.
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PH can be caused by many things. It can happen if the left side of your heart has trouble pumping blood and the arteries that take blood from your right ventricle to your lungs tighten, raising pressure in those arteries. PH can also be caused by conditions that affect the lungs or blood vessels in the lungs, such as lung cancer, air pollution and medications.
If you have Group 2 PH, your symptoms are due to a problem with the left side of your heart that affects the right side of your heart and the pulmonary arteries. Group 3 PH is caused by certain lung diseases that make your lungs’ arteries tighten and limit how much blood flows through them.
There’s no cure for pulmonary hypertension, but there are treatments to control symptoms and prevent the condition from worsening. Avoid activities that raise your blood pressure, such as sitting for long periods of time, taking hot baths or showers and high-altitude flights. Eat a healthy diet low in salt, drink plenty of water and exercise to help manage your symptoms.
Treatment
The treatment options for pulmonary hypertension vary from person to person. Your care team may prescribe medicines that reduce blood pressure in the lungs. These include calcium channel blockers, such as diltiazem (Norvasc), nifedipine (Procardia) and amlodipine (Norvasc). These medicines help relax muscles in the walls of blood vessels. They may also decrease symptoms of Raynaud’s disease (painful color change in the fingers on exposure to cold). These medicines might not work for everyone with pulmonary hypertension.
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Blood thinners, such as warfarin (Coumadin), help prevent blood clots in the lungs. Other medications that help reduce blood pressure in the lungs are tadalafil (Adcirca), macitentan (Opsuma) and sildenafil (Viagra). Sildenafil helps improve symptoms and exercise tolerance for people with pulmonary hypertension caused by connective tissue disease, but not those caused by idiopathic pulmonary hypertension. It is important to tell your care provider if you have a history of heart disease, such as coronary artery disease or valve problems, before starting this medicine.
If your condition is advanced and you aren’t getting better with medicines, a lung or heart-lung transplant might be needed. This is especially likely if your symptoms don’t go away or if you have complications such as irregular heartbeats (arrhythmias).
Even though there’s no cure for pulmonary hypertension, treatments can slow the progression of the disease and give you a better quality of life. Be sure to follow your health care professional’s advice and get regular checkups and tests. Let your provider know if you have new or changing symptoms. It is important to get enough sleep and avoid over-exerting yourself. Also, eat a healthy diet with lots of fruits, vegetables and whole grains. Limit your intake of salt, fat and sugar. And be sure to get a yearly flu shot and other vaccines that might protect you from infection. Avoid smoking, as it can make pulmonary hypertension worse. Finally, take part in any research studies that your health care provider suggests. These can help find new ways to treat and prevent pulmonary hypertension. You can learn more about Mayo Clinic clinical trials by visiting our website.
Prevention
Pulmonary hypertension (PH) is high blood pressure in the arteries of the lungs. If left untreated, it can lead to right heart failure and death. It can develop from a number of conditions, such as lung disease, heart disease and blood clots, and also from some environmental factors.
When you have PH, the blood vessels that carry oxygen-poor blood from your heart to your lungs become narrow and restricted, which raises the pressure in your pulmonary arteries. Your heart then works harder to pump blood to your lungs, and over time the right side of your heart becomes weaker. The different forms of PH include hereditable PAH, idiopathic PAH and chronic thromboembolic PAH (CTEPH).
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Treatment is aimed at improving your quality of life, preventing further complications and reducing the risk of a heart attack or stroke. Symptoms usually improve with treatment, but they may not go away completely. You can make lifestyle changes to help manage your symptoms. These include eating a healthy diet, getting regular physical activity and not smoking. A health care professional can help you choose a diet that is low in fat, cholesterol and sodium. You should also drink plenty of water and get enough sleep. If you are overweight, try to lose weight.
You can also prevent blood clots by taking a blood thinner, such as warfarin or a PDE5 inhibitor, like vardenafil or tadalafil. Be sure to talk with your health care professional before taking any over-the-counter medicines, as they can interact with these drugs and worsen your symptoms. You should also get a flu and pneumonia vaccine every year, and get recommended vaccinations for other diseases. Women of childbearing potential with PH should discuss birth control options with their health care professional, including pregnancy termination, as pregnancy can increase the severity of symptoms and lead to death.
You should also talk with your doctor if you are considering having children, as they can advise you about adoption and surrogacy options. Depending on the type of PH you have, some medications, such as endothelin receptor antagonists and riociguat, are not safe to take while pregnant.
