Pulmonary Arterial Hypertension

Pulmonary arterial hypertension is a serious condition where the small blood vessels in your lungs become narrow, making it harder for blood to flow through and causing your heart to work overtime.

Table of contents

• What is Pulmonary Arterial Hypertension?
• Symptoms
• Causes and Risk Factors
• How It’s Diagnosed
• Treatment Options
• Living With PAH

What is Pulmonary Arterial Hypertension?

Pulmonary arterial hypertension (PAH) is one form of a broader condition known as pulmonary hypertension, which is high blood pressure in the blood vessels that supply the lungs^[3]. In PAH, the small blood vessels in your lungs become narrow, and as a result, blood can’t flow through your lungs as well as it should. This leads to high blood pressure in your pulmonary arteries, which are the arteries that carry oxygen-poor blood from your heart to your lungs^[2].

When blood vessels in the lungs become thickened, narrowed, blocked or destroyed, it’s harder for blood to flow through the lungs. As a result, blood pressure goes up in the lungs^[1]. The damage makes it hard for blood to move through the lungs, and 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^[1].

Eventually, your heart gets weaker because it has to work harder to pump blood to your lungs. Without treatment, PAH can cause a cascade of problems in your heart, lungs and the rest of your body^[2]. PAH strains the right side of your heart, which pumps oxygen-poor blood to your lungs. This strain can lead to right-sided heart failure^[2].

PAH is not as common as other forms of pulmonary hypertension, including those caused by underlying heart or lung disease. Each year, about 500 to 1,000 people are diagnosed with PAH in the United States. In Western countries, about 25 per 1 million people are living with PAH^[2].

Symptoms

The symptoms of pulmonary arterial hypertension develop slowly. You may not notice them for months or even years. Symptoms get worse as the disease continues^[1]. You may not feel any symptoms early on. Most people with PAH begin to notice symptoms as the condition progresses^[2].

Pulmonary arterial hypertension symptoms include:

• Shortness of breath. It may first start during exercise and eventually happen at rest^[1]. This is the main symptom and usually starts slowly and gets worse as time goes on^[7].
• Fatigue^[2]
• Chest pressure or pain^[1]
• Dizziness or fainting^[1]
• Fast pulse or pounding heartbeat^[1]
• Blue or gray skin. Depending on skin color, these changes may be harder or easier to see^[1].
• Swelling in your feet and legs, later progressing to your belly and neck^[2]

Without treatment, PAH symptoms continue to get worse over time. You may find it harder to do your usual activities without needing to catch your breath or rest^[2]. You may notice that you can’t do some of the things you used to without feeling tired and out of breath^[7].

Causes and Risk Factors

Damage to the lining of the blood vessels in your lungs leads to pulmonary arterial hypertension. It’s not always clear what causes this damage. When there’s no clear cause, you have what’s called “idiopathic” pulmonary arterial hypertension^[2]. Over 50% of pulmonary arterial hypertension cases worldwide have no known cause^[8].

A few things can cause pulmonary arterial hypertension. Genes may play a role in why some people get it. When that’s the case, it’s called heritable pulmonary arterial hypertension. Of people that have PAH, about 15 to 20 percent have this heritable type^[7].

Known causes of PAH include associated medical conditions, genetic mutations and certain drugs^[2]. Medical conditions that may lead to the development of PAH include:

• Congenital heart disease^[2]
• HIV^[2]
• Liver disease^[2]
• Lupus^[2]
• Portal hypertension^[2]
• Infections such as schistosomiasis (snail fever)^[7]

Using drugs such as cocaine or methamphetamine, as well as cirrhosis of the liver and scleroderma can also lead to pulmonary arterial hypertension^[7].

Pulmonary arterial hypertension can affect adults at any age. It’s more common among females, who are usually diagnosed between the ages of 30 and 60. Males over age 65 who develop PAH are more likely to have severe cases^[2]. People of African and Hispanic descent are more likely to be affected^[7].

How It’s Diagnosed

Pulmonary hypertension is hard to diagnose early. 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^[9]. Correctly diagnosing pulmonary hypertension can sometimes take time because its symptoms are similar to those of many other heart and lung conditions^[6].

To diagnose pulmonary hypertension, a healthcare professional examines you and asks about your symptoms. You are usually asked questions about your medical and family history^[9]. Your doctor may ask you questions such as: Do you smoke? Does anyone in your family have heart or lung disease? When did your symptoms start? What makes your symptoms better or worse? Do your symptoms ever go away?^[7]

Tests to diagnose pulmonary hypertension may include:

• Blood tests. Blood tests can help find the cause of pulmonary hypertension. The test also may help find complications of the disease^[9].
• Chest X-ray. A chest X-ray is a picture of the heart, lungs and chest. It may be used to check for other lung conditions that can cause pulmonary hypertension^[9].
• Electrocardiogram (ECG or EKG). This simple test records the electrical activity of the heart. It shows how the heart is beating^[9].
• Echocardiogram. Sound waves create pictures of the beating heart. An echocardiogram shows how blood flows through the heart and heart valves. This test may be done to help diagnose pulmonary hypertension or to learn how treatments are working^[9]. This is an ultrasound picture of your beating heart that can check blood pressure in your pulmonary arteries^[7].
• Right heart catheterization. If an echocardiogram shows pulmonary hypertension, this test may be done to confirm the diagnosis. During this procedure, a doctor places a thin, flexible tube called a catheter into a blood vessel, usually in the neck. The tube is gently guided into the lower right heart chamber and the pulmonary artery^[9].
• CT scan, which can show enlarged pulmonary arteries or other problems in the lungs that could cause shortness of breath^[7]
• Ventilation-perfusion scan (V-Q scan), which can help find blood clots that can cause high blood pressure in your lungs^[7]

Treatment Options

Pulmonary hypertension cannot be cured, but treatment can reduce the symptoms and help you manage your condition^[6]. There’s no cure for pulmonary hypertension, but treatments are available to help you feel better, live longer and improve your quality of life^[1].

If another condition is causing pulmonary hypertension, the underlying condition should be treated first. This can sometimes prevent the pulmonary arteries being permanently damaged^[6]. Treatments for pulmonary hypertension will depend on the cause of the condition. Many times, there is no cure for pulmonary hypertension, but your provider can work with you to manage the symptoms^[8].

Treatments for pulmonary hypertension may include:

• Anticoagulant medicines to reduce the blood’s ability to thicken (clot)^[6]
• Diuretics (water tablets) to remove excess fluid from the body caused by heart failure^[6]
• Home oxygen therapy, which may be prescribed if the level of oxygen in your blood is low^[6]
• Digoxin, which can improve your symptoms by strengthening your heart muscle contractions and slowing down your heart rate^[6]

There are also a number of specialist treatments for pulmonary hypertension that help relax the arteries in the lungs and reduce the blood pressure in the lungs. These medicines may slow the progression of pulmonary hypertension, and may help to control or reduce the symptoms^[6].

Other treatments that are sometimes used are:

• Endothelin receptor antagonists, such as bosentan, ambrisentan and macitentan^[6]
• Phosphodiesterase 5 inhibitors, such as sildenafil and tadalafil^[6]
• Prostaglandins, such as epoprostenol, iloprost and treprostinil^[6]
• Soluble guanylate cyclase stimulators, such as riociguat^[6]
• Calcium channel blockers, such as nifedipine, diltiazem, nicardipine and amlodipine^[6]

Some people with pulmonary hypertension may need surgery. The types of surgery currently used are:

• Pulmonary endarterectomy, an operation to remove old blood clots from the pulmonary arteries in the lungs in people with chronic thromboembolic pulmonary hypertension^[6]
• Balloon pulmonary angioplasty, a new procedure where 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. It may be considered if pulmonary endarterectomy is not suitable, and has been shown to lower blood pressure in the lung arteries, improve breathing, and increase the ability to exercise^[6].
• Atrial septostomy, where a small hole is made in the wall between the left and right atria of the heart using a cardiac catheter, a thin, flexible tube inserted into the heart’s chambers or blood vessels. It reduces the pressure in the right side of the heart, so the heart can pump more efficiently and the blood flow to the lungs can be improved^[6].
• Transplant. In severe cases, a lung transplant or a heart-lung transplant may be needed. This type of surgery is rarely used because effective medicine is available^[6].

Living With PAH

Pulmonary arterial hypertension is a serious condition that can be life-threatening. An early diagnosis and swift treatment can help you live longer and have a better quality of life^[2]. While the current treatments showed improvements in quality of life and hemodynamic parameters (measurements of blood flow and pressure), they have shown only very limited beneficial effects on survival and disease progression^[13].

Having pulmonary hypertension can affect your ability to carry out everyday activities^[6]. It’s scary to learn you have a problem with your lungs or heart. A diagnosis of pulmonary hypertension is often life-changing, but there are treatments and ways to manage it^[19].

Managing any underlying health conditions is crucial when living with PAH and other types of pulmonary hypertension. Doing so can help slow the progression of the disease^[19]. It’s vital to monitor your symptoms and be on the lookout for any new ones. Make sure you call your health care provider if any symptoms get worse. It might mean that your condition is progressing^[19].

Self-management strategies are emphasized, including maintaining a healthy diet, staying physically active, getting adequate rest, and managing stress^[23]. Being active is good for your heart and lungs. But for some people with PAH, even light activity can make you feel very tired or short of breath. Walking is usually okay. So are swimming and stretching. Check with your doctor about which activities are best for you. Don’t strain or push yourself too hard. Stop as soon as you feel lightheadedness, fatigue, or pressure in your chest^[17].

One of the best ways to lessen fluid buildup in your body, which makes your heart work harder, is to eat less salt. You may be surprised at how much sodium is in bread and breaded foods, crackers, pre-cooked chicken breasts, condiments and bottled sauces, cheese, and even over-the-counter medicines. Check labels, and look for lower-sodium versions^[17].

Weigh yourself every day. It’s a good way to tell if your body is holding on to water. Step on the scale at the same time each day. If you’ve gained 2 pounds or more since yesterday, or more than 5 in a week, call your doctor. It may be a sign your PAH is getting worse or that you need your medications adjusted^[17].

During cold and flu season, wash your hands often to protect yourself from germs. Try not to touch your eyes, nose, and mouth. Disinfect high-traffic surfaces at home. It’s the best way to prevent the flu. You may also need a pneumonia vaccine. Both illnesses can be very serious when you have PAH. Even a cold can make some symptoms worse, like shortness of breath^[17].

Emotional support is crucial, and patients are encouraged to seek help from support groups and counseling services^[23]. The Pulmonary Hypertension Association offers practical information and support for people living with pulmonary hypertension and their friends and families^[6].