Pulmonary Hypertension

Pulmonary hypertension is a serious condition where blood pressure becomes dangerously high in the arteries between your heart and lungs, making it harder for blood to flow and forcing your heart to work much harder than normal.

Table of contents

• What is pulmonary hypertension?
• Different types of pulmonary hypertension
• Signs and symptoms
• What causes pulmonary hypertension?
• How is it diagnosed?
• Treatment options
• Living with pulmonary hypertension
• What to expect

What is pulmonary hypertension?

Pulmonary hypertension is a condition that affects the blood vessels in the lungs. It develops when the blood pressure in your lungs is higher than normal^[1]. The blood vessels in the lungs are called pulmonary arteries, and these carry oxygen-poor blood from your heart to your lungs^[2].

When the pulmonary arteries become damaged, narrowed, or blocked, blood cannot flow through them as easily. This increases the blood pressure in these arteries and causes pulmonary hypertension^[3]. The walls of the pulmonary arteries become thick and stiff, and cannot expand as well to allow blood through^[4].

The reduced blood flow makes it harder for the right side of your heart to pump blood through the arteries. Over time, this extra effort causes the heart muscle to become weak and can eventually lead to heart failure, a condition where the heart cannot pump blood effectively^[1].

About 1% of people globally have pulmonary hypertension^[3]. It is a rare but serious condition that can be life-threatening without treatment^[5].

Different types of pulmonary hypertension

Pulmonary hypertension is divided into five groups based on what causes the disease. This classification helps doctors determine the best treatment approach^[3].

Group 1: Pulmonary arterial hypertension (PAH). This group includes cases where the small blood vessels in the lungs become narrow, thick, or stiff. PAH can be idiopathic (with no known cause), inherited through families, or caused by other conditions such as HIV, connective tissue diseases like systemic sclerosis, congenital heart disease, or certain drugs and toxins^[4]. Over 50% of pulmonary arterial hypertension cases worldwide have no known cause^[3].

Group 2: Pulmonary hypertension due to left-sided heart disease. This is the most common type of pulmonary hypertension in the United States^[3]. When the left side of your heart has problems, it affects the right side of your heart and your entire pulmonary system. Blood backs up in your heart, raising the pressure in your pulmonary arteries^[4].

Group 3: Pulmonary hypertension due to lung disease or low oxygen. Certain lung problems cause the arteries in your lungs to tighten. Conditions like chronic obstructive pulmonary disease (COPD), sleep-disordered breathing, and interstitial lung disease (scarring of the lungs) can lead to this type^[4].

Group 4: Chronic thromboembolic pulmonary hypertension (CTEPH). Blood clots or scars from blood clots prevent your blood from flowing normally through your lungs. This puts more stress on the right side of your heart and raises pulmonary blood pressure^[4].

Group 5: Pulmonary hypertension with unknown or multiple causes. This includes cases related to metabolic, systemic, and blood disorders such as sickle cell disease, and other conditions^[4].

Signs and symptoms

The symptoms of pulmonary hypertension develop slowly. You may not notice them for months or even years. Symptoms get worse as the disease continues^[1]. The symptoms are often nonspecific and similar to those of other heart and lung conditions, which can make diagnosis challenging^[5].

Common symptoms include:

• Shortness of breath. This may first start during exercise and eventually happen at rest^[1].
• Tiredness or fatigue. Many patients report that feeling tired is one of the most common symptoms^[5].
• Dizziness or fainting. These symptoms can occur because your body is not getting enough oxygen-rich blood^[5].
• Chest pressure or pain. This is also called angina^[1].
• Fast pulse or pounding heartbeat. You may feel your heart racing or beating irregularly^[1].
• Blue or gray skin. Depending on skin color, these changes may be harder or easier to see^[1].
• Swelling. Fluid can build up in the legs, ankles, feet, or abdomen^[5].

If you experience chest pain that does not stop after a few minutes, or if you have heart palpitations with chest pain, shortness of breath, or feeling faint, call emergency services immediately. These could be signs of a serious problem like a blood clot in your lungs or a heart emergency^[5].

What causes pulmonary hypertension?

The cause of pulmonary hypertension is often unknown. The condition can develop on its own or be caused by another disease or condition^[1].

Some possible causes and risk factors include:

• Heart diseases, including left-sided heart failure and congenital heart disease (heart defects you are born with)^[7].
• Lung diseases such as COPD, emphysema, sleep apnea, and interstitial lung disease^[7].
• Blood clots in the lungs, also called pulmonary embolism^[7].
• Other medical conditions such as liver diseases, sickle cell disease, and connective tissue disorders like scleroderma^[7].
• Certain medicines and drugs. Some medications used to treat cancer and depression, as well as illegal drugs like cocaine, can raise your risk^[7].
• Family history and genetics. Certain genetic disorders can raise your risk of pulmonary hypertension^[7].
• Environmental factors. Being exposed to asbestos or having certain infections caused by parasites can raise your risk^[7].
• Age. The risk increases as you get older. The condition is usually diagnosed between ages 30 and 60^[7].
• Lifestyle habits. Smoking and illegal drug use can raise your risk^[7].
• Sex. Pulmonary hypertension is more common in women than in men^[7].

How is it diagnosed?

Pulmonary hypertension can be difficult to diagnose and may take years before someone gets a proper diagnosis^[7]. It is hard to diagnose early and is 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^[9].

To diagnose pulmonary hypertension, your healthcare provider will examine you and ask about your symptoms and medical history^[5]. It is important to be evaluated by a team that has expertise in pulmonary hypertension^[16].

Tests may include:

• Blood tests. These can help find the cause of pulmonary hypertension and check for complications^[9].
• Chest X-ray. This imaging test creates a picture of the heart, lungs, and chest^[9].
• Electrocardiogram (ECG or EKG). This simple test records the electrical activity of the heart^[9].
• Echocardiogram. This test uses sound waves to create pictures of the beating heart. It is recommended as the initial test in the evaluation of patients with suspected pulmonary hypertension^[9].
• Right heart catheterization. This is a procedure where a doctor places a thin, flexible tube called a catheter into a blood vessel and guides it into the right heart chamber and pulmonary artery. This test confirms the diagnosis and measures the blood pressure in the pulmonary arteries^[9]. A definitive diagnosis cannot be made on echocardiographic abnormalities alone^[14].
• CT scan or MRI. These imaging tests can help identify other conditions^[9].
• Lung function tests. These measure how well your lungs are working^[7].

Treatment options

Pulmonary hypertension cannot be cured, but treatments can reduce your symptoms and help you manage your condition^[5]. There is usually no cure for pulmonary hypertension, but your provider can work with you to manage the symptoms^[3].

If pulmonary hypertension is caused by another condition, treatments will focus on the underlying condition. If it is caused by blood clots, you may be offered medicines to prevent more clots from forming^[10].

Medicines that may be used include:

• Anticoagulants (blood thinners). These help prevent blood clots^[10].
• Diuretics (water tablets). These remove excess fluid from the body caused by heart failure^[10].
• Digoxin. This can improve your symptoms by strengthening your heart muscle contractions and slowing down your heart rate^[10].
• Oxygen therapy. If oxygen levels in your blood are low, you may need to use supplemental oxygen to maintain saturation above 90%^[11].
• Vasodilator therapy. These medicines help relax blood vessels and lower blood pressure in the pulmonary artery. They include calcium channel blockers, endothelin receptor antagonists (such as bosentan, ambrisentan, and macitentan), phosphodiesterase type 5 inhibitors (such as sildenafil and tadalafil), prostaglandins (such as epoprostenol, iloprost, and treprostinil), and soluble guanylate cyclase stimulators (such as riociguat)^[10].

It is important to note that use of vasodilator therapies in patients with pulmonary hypertension due to lung disease or left heart disease is potentially harmful and not recommended^[14].

Surgical procedures that may be used include:

• Pulmonary endarterectomy. This is an operation to remove old blood clots from the pulmonary arteries in people with chronic thromboembolic pulmonary hypertension^[10].
• Balloon pulmonary angioplasty. A tiny balloon is guided into the arteries and inflated for a few seconds to push the blockage aside and restore blood flow to the lung^[10].
• Atrial septostomy. A small hole is made in the wall between the left and right chambers of the heart to reduce pressure^[10].
• Transplant. In severe cases, a lung transplant or a heart-lung transplant may be needed^[10].

Results from right heart catheterization should be obtained before initiating treatment with vasodilator therapy in patients with pulmonary arterial hypertension^[14].

Living with 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^[21].

Monitor your condition. Talk to your doctor about new or concerning symptoms. You may need regular tests to monitor your ability to exercise, blood tests to check organ function, and imaging tests^[21]. Weigh yourself every day at the same time. If you have gained 2 pounds or more since yesterday, or more than 5 pounds in a week, call your doctor, as this may be a sign your condition is getting worse^[20].

Conserve your energy. Fatigue and shortness of breath frequently accompany pulmonary hypertension. Prioritize your daily tasks, breaking up tiring activities into smaller segments, and schedule mandatory rest periods between tasks^[22]. Organize your home for energy efficiency by storing frequently used items within easy reach and installing adaptive equipment such as grab bars and shower benches^[22].

Stay active safely. While stringent bed rest was once the standard, gentle exercise may help strengthen muscles and improve overall well-being when done carefully^[22]. Walking is usually safe, as are swimming and stretching. Check with your doctor about which activities are best for you. Stop as soon as you feel lightheadedness, fatigue, or pressure in your chest^[20].

Eat a heart-healthy diet. One of the best ways to lessen fluid buildup in your body is to eat less salt. Check labels and look for lower-sodium versions of foods^[20]. Track how much fluid you drink throughout the day, especially if your doctor limits you^[20].

Protect yourself from infections. Patients with pulmonary hypertension should receive seasonal influenza vaccination and age-appropriate pneumococcal vaccination, unless contraindicated^[14]. Even a cold can make some symptoms worse. During cold and flu season, wash your hands often and try not to touch your eyes, nose, and mouth^[20].

Avoid certain situations. Stay out of hot tubs, saunas, and steam rooms, as the heat can cause your blood pressure to drop too low^[20]. Avoid bending over too often, as this could trigger symptoms^[20]. Tell your doctor about travel plans, as you may need oxygen therapy if you are flying or heading to the mountains^[20].

Use birth control. Pregnancy is risky for women with pulmonary hypertension. It causes changes in your body that can lead to serious or fatal health problems for you and your unborn baby. Talk to your doctor about safe forms of contraception^[20].

Ask for help. Accept offers of help from friends and family for tasks like making meals, cleaning, and yard work. Leverage online shopping to order pantry staples and medications for delivery^[22].

What to expect

Pulmonary hypertension usually gets worse over time. Left untreated, it may cause heart failure, which can be fatal^[5]. Pulmonary hypertension can get worse over time and lead to serious problems, including anemia, irregular heartbeats, blood clots in the pulmonary arteries, bleeding in the lungs, heart failure, liver damage, fluid collection around the heart, and serious pregnancy complications^[21].

The outlook for pulmonary hypertension varies, depending on factors such as what is causing it, how quickly it is diagnosed, how advanced your symptoms are, and whether you have another underlying health condition^[5].

Early diagnosis and treatment can help you enjoy a better quality of life^[4]. Taking steps to follow your treatment plan, get regular care, and learn how to monitor your condition can slow down the progression of the disease and may improve your condition^[21].