Overview

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

In one form of pulmonary hypertension, called pulmonary arterial hypertension (PAH), blood vessels in your lungs are narrowed, blocked or destroyed. The damage slows blood flow through your lungs, and blood pressure in the lung arteries rises. Your heart must work harder to pump blood through your lungs. The extra effort eventually causes your heart muscle to become weak and fail.

In some people, pulmonary hypertension slowly gets worse and can be life-threatening. Although there’s no cure for some types of pulmonary hypertension, treatment can help reduce symptoms and improve your quality of life.

Symptoms

The signs and 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 (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 chambers (atria) and two lower chambers (ventricles). 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 blood normally flows easily through blood vessels in your lungs (pulmonary arteries, capillaries and veins) to the left side of your heart.

However, changes in the cells that line your pulmonary arteries can cause the walls of the arteries to become stiff, swollen and thick. These changes may slow down or block blood flow through the lungs, causing pulmonary hypertension.

Diagnosis

Pulmonary hypertension is hard to diagnose early because it’s not often detected in a routine physical exam. Even when the condition is more advanced, its signs and symptoms are similar to those of other heart and lung conditions.

Your doctor will perform a physical exam and talk to you about your signs and symptoms. You’ll be asked questions about your medical and family history.

Your doctor will order tests to help diagnose pulmonary hypertension and determine its cause. Tests for pulmonary hypertension may include:

Blood tests. Blood tests can help your doctor determine the cause of pulmonary hypertension or look for signs of complications.
Chest X-ray. A chest X-ray creates pictures of your heart, lungs and chest. It can show enlargement of the right ventricle of the heart or the pulmonary arteries, which can occur in pulmonary hypertension. Your doctor may also use a chest X-ray to check for other lung conditions that can cause pulmonary hypertension.
Electrocardiogram (ECG). This noninvasive test shows your heart’s electrical patterns and can detect abnormal heartbeats. An ECG may also reveal signs of right ventricle enlargement or strain.
Echocardiogram. Sound waves can create moving images of the beating heart. An echocardiogram lets your doctor see how well your heart and its valves are working. It can be used to determine the size and thickness of the right ventricle, and to measure the pressure in your pulmonary arteries.

Sometimes, an echocardiogram is done while you exercise on a stationary bike or treadmill to understand how well your heart works during activity. You may be asked to wear a mask that checks how well your heart and lungs use oxygen and carbon dioxide.

An echocardiogram may also be done after diagnosis to assess how your treatments are working.

Right heart catheterization. If an echocardiogram reveals pulmonary hypertension, you’ll likely have a right heart catheterization to confirm the diagnosis.

During this 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 of the heart. It’s also used to see what effect different medications may have on your pulmonary hypertension.

Your doctor might also order one or more of the following tests to check the condition of your lungs and pulmonary arteries and further determine the cause of pulmonary hypertension:

Computerized tomography (CT). This imaging test creates cross-sectional pictures of the bones, blood vessels and soft tissues inside your body. A CT scan can show the heart’s size, spot blood clots in the lungs’ arteries, and look closely for lung diseases that might lead to pulmonary hypertension, such as COPD or pulmonary fibrosis.

Sometimes, a special dye, called contrast, is injected into your blood vessels before the CT scan (CT angiography). The dye helps your arteries show up more clearly on the images.

Magnetic resonance imaging (MRI). An MRI scan uses a magnetic field and pulses of radio wave energy to make pictures of the body. Your doctor may order this test to check the right ventricle’s function and blood flow in the lung’s arteries.
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.
Polysomnogram. This test measures your brain activity, heart rate, blood pressure, oxygen levels and other factors while you sleep. It can help diagnose a sleep disorder such as obstructive sleep apnea, which can cause pulmonary hypertension.
Ventilation/perfusion (V/Q). In this test, a tracer is injected into a vein in your arm. The tracer shows blood flow and air to your lungs. A V/Q test can determine whether blood clots are causing symptoms of pulmonary hypertension.
Open-lung biopsy. Rarely, a doctor might recommend an open-lung biopsy to check for a possible cause of pulmonary hypertension. An open-lung biopsy is a type of surgery in which a small sample of tissue is removed from your lungs while you are under general anesthesia.

Treatment

There’s no cure for pulmonary hypertension, but your doctors can prescribe treatments to help you manage your condition. Treatment may help improve your symptoms and slow the progress of pulmonary hypertension.

It often takes some time to find the most appropriate treatment for pulmonary hypertension. The treatments are often complex and require extensive follow-up care.

When pulmonary hypertension is caused by another condition, your doctor will treat the underlying cause whenever possible.

Risk factors

Growing older can increase your risk of developing pulmonary hypertension. The condition is more often diagnosed in people ages 30 to 60. However, idiopathic PAH is more common in younger adults.

Other things that can raise your risk of pulmonary hypertension include:

A family history of the condition
Being overweight
Blood-clotting disorders or a family history of blood clots in the lungs
Exposure to asbestos
Genetic disorders, including congenital heart disease
Living at a high altitude
Use of certain weight-loss drugs
Use of illegal drugs such as cocaine
Use of selective serotonin reuptake inhibitors (SSRIs), used to treat depression and anxiety