Pulmonary Hypertension

What is Pulmonary Hypertension?

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

Pulmonary hypertension begins when tiny arteries in your lungs, called pulmonary arteries, and capillaries become narrowed, blocked or destroyed. This makes it harder for blood to flow through your lungs, and raises pressure within your lungs’ arteries. As the pressure builds, your heart’s lower right chamber (right ventricle) must work harder to pump blood through your lungs, eventually causing your heart muscle to weaken and eventually fail.

Pulmonary hypertension is a serious illness that becomes progressively worse and is sometimes fatal. Although pulmonary hypertension isn’t curable, treatments are available that can help lessen symptoms and improve your quality of life.

Symptoms

The signs and symptoms of pulmonary hypertension in its early stages may not be noticeable for months or even years. As the disease progresses, symptoms become worse.

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 and two lower chambers. 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 oxygen-rich blood then flows through blood vessels in your lungs (pulmonary arteries, capillaries and veins) to the left side of your heart.

Ordinarily, the blood flows easily through the vessels in your lungs, so blood pressure is usually much lower in your lungs. With pulmonary hypertension, the rise in blood pressure is caused by changes in the cells that line your pulmonary arteries. These changes cause extra tissue to form, eventually narrowing or completely blocking the blood vessels, making the arteries stiff and narrow. This makes it harder for blood to flow, raising the blood pressure in the pulmonary arteries.

Idiopathic pulmonary hypertension

When an underlying cause for high blood pressure in the lungs can’t be found, the condition is called idiopathic pulmonary hypertension.

Some people with IPH may have a gene that’s a risk factor for developing pulmonary hypertension. But in most people with idiopathic pulmonary hypertension, there is no recognized cause of their pulmonary hypertension.

Secondary pulmonary hypertension

Pulmonary hypertension that’s caused by another medical problem is called secondary pulmonary hypertension. This type of pulmonary hypertension is more common than is idiopathic pulmonary hypertension. Causes of secondary pulmonary hypertension include:

• Blood clots in the lungs (pulmonary emboli)
• Chronic obstructive pulmonary diseases, such as emphysema
• Connective tissue disorders, such as scleroderma or lupus
• Sleep apnea and other sleep disorders
• Heart abnormalities you’re born with (congenital heart defects)
• Sickle cell anemia
• Chronic liver disease (cirrhosis)
• AIDS
• Lung diseases such as pulmonary fibrosis, a condition that causes scarring in the tissue between the lungs’ air sacs (interstitium)
• Left-sided heart failure
• Living at altitudes higher than 8,000 feet (2,438 meters)
• Climbing or hiking to altitudes higher than 8,000 feet (2,438 meters) without acclimating first
• Use of certain stimulant drugs, such as cocaine

Eisenmenger syndrome and pulmonary hypertension

Eisenmenger syndrome, a type of congenital heart defect, causes pulmonary hypertension. It is most commonly caused by a large hole in your heart between the two lower heart chambers (ventricles), called a ventricular septal defect (VSD). This hole in your heart causes blood to circulate abnormally 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.

Tests and Diagnosis

Pulmonary hypertension is hard to diagnose early because it’s not often detected in a routine physical exam. Even when the disease is more advanced, its signs and symptoms are similar to those of other heart and lung conditions. Your doctor may do one or more tests to rule out other possible reasons for your condition. The first tests you’ll have to diagnose pulmonary hypertension include:

• Blood tests: Your doctor may order blood tests to check for certain substances in your blood that may show you have pulmonary hypertension or its complications.
• Chest X-ray: This test may be able to check for pulmonary hypertension if your pulmonary arteries or the right ventricle of your heart is enlarged. The X-ray will appear normal in about one-third of people who have pulmonary hypertension.
• Doppler echocardiogram: Your doctor may first suspect you have pulmonary hypertension based on the results of this test. This noninvasive test uses sound waves that allow your doctor to see your heart without making an incision. During the procedure, a small, plastic instrument called a transducer is placed on your chest. It collects reflected sound waves (echoes) from your heart and transmits them to a machine that uses the sound wave patterns to compose images of your beating heart on a monitor.

These images show how well your heart is functioning, and recorded pictures allow your doctor to measure the size and thickness of your heart muscle. Sometimes your doctor will recommend an exercise echocardiogram to help determine how well your heart works under stress. In that case, you’ll have an echocardiogram before exercising on a stationary bike or treadmill and another test immediately afterward.

• Trans esophageal echocardiogram: If it’s difficult to get a clear picture of your heart and lungs with a standard echocardiogram, your doctor may recommend a trans esophageal echocardiogram. In this procedure, a flexible tube containing a transducer is guided down your throat and into your esophagus after using a numbing spray in the back of your throat. From here, the transducer can get detailed images of your heart.
• Right heart catheterization: After you’ve had an echocardiogram, if your doctor thinks you have pulmonary hypertension, you’ll likely have a right heart catheterization. This test is often the most reliable way of diagnosing pulmonary hypertension. During the 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. It’s also used to see what effect different medications may have on your pulmonary hypertension.

Right heart catheterization is usually performed during local anesthesia and sedation in a hospital setting. You often can go home soon after the procedure. You’ll need someone to drive you home after the test.

Your pulmonologist may order additional tests to check the condition of your lungs and pulmonary arteries, including:

• 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.
• Perfusion lung scan: This test uses small amounts of radioactive substances (radioisotopes) to study blood flow (perfusion) in your lungs. The radioisotopes are injected into a vein in your arm. Immediately afterward, a special camera (gamma camera) takes pictures of blood flow in your lungs’ blood vessels. A lung scan can then be used to determine whether blood clots are causing symptoms of pulmonary hypertension.

A perfusion lung scan is usually performed with another test, known as a ventilation scan. In this test, you inhale a small amount of radioactive substance while a gamma camera records the movement of air into your lungs. The two-test combination is known as a ventilation-perfusion (V/Q) scan.

• Computerized tomography (CT) scan: A CT scan allows your doctor to see your organs in 2-D “slices.” In this test, you’ll lie in a machine that takes images of your lungs so that your doctors can see a cross-section of them. You might also be given a medication that makes the images of your lungs show up more clearly.
• Magnetic resonance imaging (MRI): This test, which uses no X-rays, is sometimes used to get images of the blood vessels in your lungs. A computer creates tissue “slices” from data generated by a powerful magnetic field and radio waves. An MRI can’t, however, measure artery pressure — a procedure that’s necessary to check the effectiveness of any medications you’re taking to control IPH.

Treatment

It often takes some time to find the best treatment for pulmonary hypertension. The treatments are often complex and require extensive follow-up care. Your doctor may also need to change your treatment if it’s no longer effective. When pulmonary hypertension is caused by another condition, your doctor will treat the underlying cause whenever possible.

Lifestyle and home remedies

Although medical treatment can’t cure pulmonary hypertension, it can lessen symptoms. Lifestyle changes also can help improve your condition. You should:

• Get plenty of rest: Resting can reduce the fatigue that may come from having pulmonary hypertension.
• Stay as active as possible: Even the mildest forms of activity may be too exhausting for some people with pulmonary hypertension. For others, moderate exercise such as walking may be beneficial, and using oxygen during exercise may be especially helpful. But first, talk to your doctor about specific exercise restrictions. In most cases, it’s recommended that you not lift more than 50 pounds (22.7 kilograms). 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 lung health 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 becoming pregnant or using birth control pills: If you’re a woman of childbearing age, avoid becoming pregnant. Pregnancy can be life-threatening for both you and your baby. Also avoid using birth control pills, which can increase your risk of blood clots. Talk to your doctor about alternative forms of birth control.
• Avoid traveling to or living 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 may recommend that you move to a lower elevation.
• 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 cause fainting or even death. You should also avoid activities that cause prolonged straining, such as lifting heavy objects or weights.
• Find ways to reduce stress: These can range from yoga, meditation and biofeedback to warm baths, music or a good book. Try to allow at least 30 minutes a day for an activity you find relaxing. Many people with pulmonary hypertension find that simply reducing stress can greatly improve the quality of their lives.
• Follow a nutritious diet and stay at a healthy weight: It’s likely your doctor will recommend limiting the amount of salt in your diet to minimize swelling of your body’s tissues (edema). Most experts agree that you should eat no more than 1,500 to 2,400 milligrams of salt a day. Keep in mind that processed foods often are high in salt, so it’s important to check labels carefully.