Pulmonary hypertension (PH) isn’t always easy to spot. In its early stages, it often flies under the radar — even during a routine physical exam. Many of its symptoms, like shortness of breath or fatigue, can be mistaken for signs of other lung or heart conditions. As a result, people with PH are sometimes misdiagnosed at first, delaying the right treatment.

PH may take years to diagnose correctly. In a small study of people diagnosed with one type of PH — idiopathic pulmonary arterial hypertension — it took almost four years on average from first experiencing symptoms to getting a diagnosis. This type of PH has no known cause (“idiopathic” means the cause is unknown). It affects the arteries in the lungs, making it harder for the heart to pump and keep normal blood flow through the lungs.

Several types of doctors can treat PH. These include pulmonologists (lung doctors), cardiologists (heart doctors), and rheumatologists (joint or immune system doctors). They have special training and experience in managing this condition.

What Tests Are Used To Diagnose Pulmonary Hypertension?

No single pulmonary hypertension test can confirm the condition, so your healthcare provider will likely order a series of tests to get a clear picture. Some tests may be repeated to get an accurate idea of what is causing high blood pressure in the pulmonary arteries. After you’re diagnosed with PH, your healthcare provider might assign a functional class (how severely the disease affects your quality of life) to your PH based on your symptoms and how they affect your daily life.

Medical History

Your healthcare provider will take a thorough history, asking for specific details about symptoms over time. They’ll likely ask whether anyone in your family has PH, since a family history of the condition is a risk factor for developing it. They’ll ask about any prescribed medications or recreational drug use. The information from your medical history can help your provider decide if you might have PH or another condition.

Physical Exam

Your healthcare provider will carefully examine you, listening to your heart and lungs. They will check for signs of swelling, changes in skin color, or any other symptoms of PH. A physical exam might show PH or another health problem.

Electrocardiogram

If your healthcare provider thinks you might have PH, they may start with an electrocardiogram (ECG or EKG). This test uses small patches on your skin to record your heartbeat. ECGs are painless, quick, and inexpensive.

A standard ECG uses 12 different patches with electrodes — small, sticky sensors that detect the heart’s electrical signals — attached to leads. The leads go to a machine that senses electrical impulses in the heart and records the pattern of heartbeats. A healthcare provider can use the results of an ECG test to determine if the right side of the heart is enlarged and to evaluate overall heart function. If the ECG shows signs that you might have PH, your healthcare provider will order more tests.

Echocardiogram

An echocardiogram is an imaging test that’s important for the diagnosis of PH. For this procedure, a healthcare worker places small electrodes on your chest to monitor your heartbeat and uses a handheld device called a transducer to create images of the heart.

Echocardiograms help healthcare providers diagnose PH, and they’re also used to diagnose other heart conditions. They show how the heart is working and whether blood flow through the heart and lungs is normal.

Cardiac Catheterization

Cardiac catheterization, or right-heart catheterization, is considered the gold-standard test for diagnosing PH. It’s used to measure blood pressure in the heart and lungs and to find any unusual connections inside the heart. Cardiac catheterization is used to confirm whether someone has PH.

Before cardiac catheterization, you’ll be given medicine to help you sleep during the procedure. Your healthcare provider will use a needle to make a small hole in a blood vessel in your arm, groin, or neck. Then, they’ll insert a thin tube into the blood vessel and guide it into your heart.

Right heart catheterization is the most accurate way to measure pressure in the pulmonary arteries and assess blood flow through the right side of the heart and lungs. Unlike other tests, it directly measures that pressure and gives your care team the clearest picture of how your heart and lungs are working. It’s the only way to confirm the diagnosis and help decide on the best treatment. This procedure is done in the hospital, where your care team can closely monitor you.

Cardiopulmonary Exercise Testing

Your healthcare provider may ask you to do a cardiopulmonary exercise test to see how well your heart and lungs work when you’re active. They may ask you to walk on a treadmill or ride a bike for several minutes while they measure your oxygen levels and several other variables. The exercise test helps show how much activity you can handle and how severely PH affects your ability to exercise.

Six-Minute Walk Test

The six-minute walk test checks how far you can walk in six minutes on flat ground. It helps your healthcare provider understand how well your heart and lungs are working during activity and how much physical activity your body can manage during that time.

Pulmonary Function Testing

Pulmonary function testing (PFT) measures your lung function. During a spirometry PFT test, you breathe into a tube that measures how much air you can blow in one large breath and in one second. This test checks different parts of how your lungs work, such as how much air you can blow out and how fast. These measurements can be used to check for lung problems and find out how well your lungs exchange oxygen.

Imaging Tests

Imaging tests are painless and noninvasive. They help healthcare providers see the heart and lungs and check for any problems with their structure or how they work. Imaging tests used to look for PH include chest X-rays, CT scans, MRI, and an echocardiogram.

Blood Tests

Examining your blood in a lab can help reveal important clues about PH. For example, your healthcare provider may order an HIV screening, since HIV infection is a possible cause of PH. They may also order a complete metabolic panel — a blood test that checks how well your body’s metabolism is doing its job. It looks at your blood sugar, calcium levels, and how well your liver and kidneys are working, and can show if something isn’t right with those organs.

Blood tests for PH can also examine:

• Arterial blood gas levels — Show the level of oxygen in your blood
• Thyroid hormone levels — Indicate whether your thyroid is functioning normally
• Brain natriuretic peptide — Can indicate heart failure if levels are high

Ventilation/Perfusion Scan

Also called a V/Q or nuclear scan, a ventilation/perfusion scan involves an injection of radioactive material followed by chest X-rays. A V/Q scan helps find blood clots that may be blocking blood flow in the pulmonary arteries.

Sleep Study

Your healthcare provider may ask you to undergo polysomnography, also called a sleep study or overnight oximetry, if they think you have obstructive sleep apnea, a potential cause of PH. A sleep study can find out whether you have low levels of oxygen at night while you’re sleeping.

Ruling Out Other Conditions

The process of ruling out similar conditions is called a differential diagnosis. PH has symptoms in common with other cardiovascular and lung diseases, such as asthma.

When PH is caused by another disease, this is known as associated PH. Some of the conditions that can cause PH include:

• Left-sided heart disease, such as left-sided heart failure
• Lung disease, such as chronic obstructive pulmonary disease (COPD)
• Lung scarring, or pulmonary fibrosis
• Sleep apnea
• Kidney disease
• Infections, such as HIV
• Autoimmune conditions, including lupus and scleroderma

Your healthcare provider may be able to quickly rule out many of these conditions based on your medical and family history or simple blood tests. Other conditions may require time and repeated tests before they can be confirmed or ruled out.

Having other health problems along with PH can make it harder for your provider to figure out exactly what you have, and you might get more than one diagnosis. However, once your healthcare provider has a full picture of your PH and what may be causing it, they can make better treatment decisions for you.

Find Your Team

On myPHteam, the social network for people with pulmonary hypertension and their loved ones, members come together to ask questions, give advice, and share their stories with those who understand life with pulmonary hypertension.

Have you been diagnosed with pulmonary hypertension? Share in the comments below, or start a conversation with others on your Activities page.