Pulmonary hypertension (PH) can be difficult to diagnose. Early on, signs of the condition are unlikely to be detected during a regular physical exam. Additionally, doctors often miss symptoms of PH or misinterpret them as symptoms of another lung or heart disease. People with pulmonary hypertension often are misdiagnosed before eventually receiving a correct diagnosis.

Pulmonary hypertension 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 the onset of first symptoms to diagnosis.

Pulmonary hypertension is usually diagnosed and treated by a PH specialist. Many types of doctors have training and experience that enables them to specialize in managing pulmonary hypertension, including pulmonologists, cardiologists, and rheumatologists.

How Is Pulmonary Hypertension Diagnosed?

Doctors use many tests to diagnose pulmonary hypertension. Some tests may be repeated to get an accurate idea of what is causing high blood pressure in the pulmonary arteries. During the diagnosis process, the doctor will categorize the type and functional class of your PH.

Medical History

Your doctor will take a thorough history, asking for specific details about symptoms over time. They’ll likely ask whether anyone in your family has pulmonary hypertension, since a family history of the condition is a risk factor for developing it. They’ll ask about any prescribed medications or illicit drugs you’ve used. A clear picture may emerge from the medical history that will help a doctor strengthen the suspicion of PH or rule out other conditions.

Physical Exam

The doctor will carefully examine you, listening to your heart and lungs and checking for swelling, changes in skin color, or any other symptoms of PH. The results of the physical exam may suggest pulmonary hypertension or another condition.

Electrocardiogram

An electrocardiogram (ECG or EKG) is often one of the first tests doctors perform when they suspect pulmonary hypertension. This test monitors your heartbeat using patches that attach to the skin. ECGs are painless, quick, and inexpensive.

A standard ECG uses 12 different patches with electrodes attached to leads. The leads go to a machine that senses electrical impulses in the heart and records the pattern of heartbeats. A doctor 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 strengthens your doctor’s suspicion of pulmonary hypertension, they’ll order more tests.

Echocardiogram

An echocardiogram is an imaging test that’s important for the diagnosis of PH. For this procedure, a health care worker places small electrodes directly on your chest. The electrodes work to capture an image of the heart. The test also assesses blood pressure and general heart function.

Echocardiograms help health care providers diagnose PH, and they’re also used to diagnose other heart conditions. Echocardiograms are also used to monitor a person’s heart and the progression of their PH over time.

Cardiac Catheterization

Cardiac catheterization, or right-heart catheterization, is considered a gold-standard test for diagnosing PH. It’s used to measure the blood pressure within the heart and pulmonary arteries and to detect abnormal connections between the right and left sides of the heart. Cardiac catheterization is used to confirm whether or not a person definitely has PH.

Before cardiac catheterization, you’ll be sedated. The doctor will use a needle to make a hole in a blood vessel in your arm, groin, or neck. They’ll then introduce a thin tube into the blood vessel and guide it into the heart.

Cardiopulmonary Exercise Testing

You may be asked to walk on a treadmill or ride a bike for several minutes while your oxygen levels and several other variables are measured. The exercise test is useful for determining your ability to exercise and the severity of any exercise limitations related to PH.

Six-Minute Walk Test

The six-minute walk test is an exercise test in which a person tries to walk for six minutes on flat ground. It’s used to help evaluate a person’s exercise tolerance level.

Pulmonary Function Testing

Pulmonary function testing (PFT) measures lung function. During a PFT test, you breathe into a tube that evaluates lung function by monitoring how much air you are able to blow during a large breath or during 1 second. The doctor uses these measurements to calculate forced expiratory volume (FEV1) and forced expiratory capacity. FEV1 scores can be used to check for lung problems and find out how effectively your lungs exchange oxygen.

Imaging Tests

Imaging tests help doctors visualize and diagnose problems with the structure of the heart and lungs. Imaging tests are painless and noninvasive. Imaging tests used to diagnose pulmonary hypertension include chest X-rays, CT, MRI, and echocardiogram.

Blood Tests

Blood samples tested in the lab can reveal many important clues about pulmonary hypertension. Your doctor may order an HIV screening, as HIV infection is one possible cause of pulmonary hypertension. A complete metabolic panel can reveal liver or kidney dysfunction.

Blood tests for PH can also examine:

• Arterial blood gas — Shows the levels of oxygen and carbon dioxide in the blood
• Thyroid hormones — Indicates whether the thyroid is functioning normally
• Brain natriuretic peptide — Can indicate heart failure when levels are high
• Autoantibodies — Looks for proteins made by the body in autoimmune illnesses, such as scleroderma and lupus, which can cause PH

Ventilation/Perfusion Scan

Also called a V/Q or nuclear scan, a ventilation/perfusion scan involves an injection of radioisotope followed by chest X-rays. A V/Q scan can help identify blood clots in the pulmonary arteries.

Sleep Study

The doctor may ask you to undergo polysomnography — also called a sleep study or overnight oximetry — if obstructive sleep apnea is suspected. A sleep study can determine whether you have low levels of oxygen at night while you are sleeping.

Ruling Out Other Conditions

The process of ruling out similar conditions is referred to as differential diagnosis. Pulmonary hypertension has symptoms in common with cardiovascular disease and lung disease, such as asthma. PH can be caused by medications or illicit drugs.

When pulmonary hypertension is caused by another disease, this is known as associated PH. Conditions that can cause associated PH include:

• Heart disease, such as coronary artery disease
• Lung disease, such as chronic obstructive pulmonary disease
• Liver or kidney failure
• Infections, such as HIV
• Autoimmune conditions, including lupus and scleroderma

Your doctor may be able to quickly rule out many of these conditions based on your medical and family history or simple blood tests. Other disorders may require time and repeated tests before they can be confirmed or ruled out. The presence of other diseases in addition to pulmonary hypertension may complicate the differential diagnosis and eventually result in multiple diagnoses.

Find Your Team

On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 50,000 members come together to ask questions, give advice, and share their stories with those who understand life with PH.

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