What is PH?

What is Pulmonary Hypertension (PH)?

Pulmonary hypertension (PH) is a serious condition that affects the blood vessels in the lungs. The term literally means high blood pressure in the lungs. When pressure in these blood vessels becomes too high, the heart has to work harder to pump blood through the lungs. Over time, this can put significant strain on the heart and, if left untreated, can lead to heart failure and can be life-threatening.

PH can be difficult to recognize because its symptoms such as shortness of breath, dizziness, fatigue, and reduced ability to exercise can look like other more common conditions, including asthma or COPD. Getting an accurate diagnosis is an important first step toward receiving appropriate care.

There is currently no cure for pulmonary hypertension, but treatment has come a long way. Today, there are multiple FDA-approved therapies that can help manage the disease, improve symptoms and quality of life, and for many patients, slow its progression.

Early diagnosis and treatment matter. Without appropriate treatment, pulmonary hypertension can be a life-threatening disease. Continued research and participation in clinical trials are essential to developing better treatments and, ultimately, finding a cure.

Every clinical trial participant helps move PH research forward.

Symptoms of Pulmonary Hypertension

The symptoms of pulmonary hypertension (PH) can be similar across the different types of the disease. They may start out mild and become more noticeable or severe as PH progresses.

Symptoms may include:

Fatigue: Feeling unusually tired or having less energy than usual.

Shortness of breath: At first, you may notice it mainly during exercise or physical activity. As PH progresses, you may become short of breath with everyday activities or even while resting.

Chest pain or pressure: You may feel discomfort, pain, or pressure in your chest.

Fast or irregular heartbeat: You may notice your heart racing, pounding, fluttering, or skipping beats. These sensations are called palpitations and may be caused by an irregular heart rhythm, or arrhythmia.

Dizziness or fainting: Feeling lightheaded or actually passing out, especially during physical activity. Fainting is also called syncope.

Blue or gray lips or skin: This can be a sign that your blood is not carrying enough oxygen and is called cyanosis.

Swelling: Fluid can build up in the legs, ankles, or feet, causing swelling known as edema.

Coughing up blood: In some cases, PH can cause bleeding in the lungs, which may result in coughing up blood. This is called hemoptysis.

When PH Becomes More Advanced

As pulmonary hypertension becomes more severe, symptoms may occur with less activity and can eventually occur even at rest. People with advanced PH may experience significant difficulty breathing, a racing or irregular heartbeat, dizziness, or fainting.

These symptoms can have many different causes. Having one or more of these symptoms does not necessarily mean you have pulmonary hypertension. However, if you experience persistent or unexplained symptoms especially shortness of breath, chest pain, dizziness, or fainting, it is important to talk with a healthcare professional.

Recognizing the symptoms and getting an accurate diagnosis can be an important first step toward appropriate care and treatment.

Who Gets Pulmonary Hypertension?

Pulmonary hypertension (PH) can affect people of any age, race, or ethnic background. While anyone can develop PH, certain health conditions, genetic factors, medications, and environmental exposures may increase a person’s risk.

Some factors associated with a higher risk of developing PH include:

Family history: Some forms of PH can run in families and may be linked to changes in certain genes.

Sex: Idiopathic pulmonary arterial hypertension (PAH) and heritable PAH are more common in women than in men.

Pregnancy: Pregnancy can place additional stress on the heart and lungs and may increase risk or worsen PH in some people.

Living at high altitude: Long-term exposure to high altitudes, where oxygen levels are lower, may increase the risk of developing or worsening PH.

Other health conditions: PH can be associated with conditions such as congenital heart disease, lung disease, liver disease, schistosomiasis, and connective tissue diseases such as scleroderma.

Certain drugs and toxins: Exposure to certain substances, including methamphetamines and some appetite-suppressant drugs, has been associated with an increased risk of PH. The combination of fenfluramine and phentermine (“fen-phen”), which was withdrawn from the U.S. market, is one well-known example.

Having one or more of these risk factors does not mean that you will develop pulmonary hypertension. Understanding your risk factors can, however, help you and your healthcare team recognize possible symptoms and determine when further evaluation may be appropriate.

Pulmonary Hypertension Diagnosis

Pulmonary hypertension (PH) can be difficult to diagnose in a routine medical exam because the most common symptoms of PH are also associated with many other conditions. If your doctor suspects that you have PH, he or she will want to review your medical and family history, perform a physical exam and perform one or more diagnostic tests.

Preliminary Tests for Pulmonary Hypertension

If your healthcare team suspects you may have pulmonary hypertension (PH), they may recommend several tests to look for signs of the disease and understand how your heart and lungs are working.

These tests can help your healthcare team determine whether additional testing is needed. If the results suggest PH, your doctor may recommend a right-heart catheterization, which is the gold-standard test used to confirm a PH diagnosis.

Preliminary tests may include:

Blood tests: Can provide information about your overall health and help identify other conditions that may be causing or contributing to your symptoms.

Chest X-ray: Can help doctors look at the heart and lungs for signs that may be associated with PH or other lung conditions.

Electrocardiogram (ECG or EKG): Records the heart’s electrical activity and can show changes in heart rate or rhythm.

Echocardiogram: Uses sound waves to create pictures of the heart and can help doctors see how well the heart is working and look for signs that may suggest PH.

Pulmonary function tests: Measure how well your lungs are working, including how much air you can move in and out of your lungs.

Exercise tolerance test (Six-Minute Walk Test): Measures how far you can walk in six minutes while your healthcare team monitors how your body responds to exercise.

Ventilation/Perfusion (V/Q) scan: A specialized imaging test that looks at airflow and blood flow in the lungs. It can help doctors determine whether blood clots or other problems with blood flow may be contributing to PH.

Confirming a Diagnosis

These tests can help point your healthcare team toward a possible diagnosis, but they do not by themselves confirm pulmonary hypertension. A right-heart catheterization (RHC) directly measures the pressure inside the heart and pulmonary arteries and is used to confirm the diagnosis.

The Gold Standard for a PH Diagnosis

Right-Heart Catheterization

If earlier tests suggest that you may have pulmonary hypertension (PH), your doctor may recommend a right-heart catheterization (RHC), often called a “right heart cath.”

A RHC is the definitive test used to confirm a diagnosis of pulmonary hypertension. Unlike other tests, it directly measures the pressure inside the heart and pulmonary arteries and provides important information about how well the heart and lungs are working.

During the procedure, your doctor guides a thin, flexible tube called a catheter through a vein, usually in the neck, arm, or groin. The catheter is carefully guided into the right side of the heart and pulmonary arteries. The catheter allows your medical team to measure pressures and other important information about blood flow through your heart and lungs.

A RHC is an important part of evaluating and diagnosing PH and can also help your healthcare team determine the most appropriate treatment.

Acute Vasodilator Challenge

An acute vasodilator challenge, sometimes called a vasodilator study, may be performed during a right-heart catheterization for certain people with pulmonary arterial hypertension (PAH).

The purpose of this test is to see how the pulmonary blood vessels respond to medication that temporarily relaxes and widens them. The results can help doctors identify a small group of patients who may benefit from calcium channel blockers (CCBs).

During the test, a short-acting medicine is given while the catheter remains in place. Your medical team carefully monitors your blood pressure, heart rate, and pressures in your heart and lungs to see how your body responds.

The test is stopped when an appropriate response is observed or if side effects make it unsafe or uncomfortable to continue.

Not everyone with PH needs an acute vasodilator challenge. Your PH specialist will determine whether this test is appropriate based on your type of PH and other factors.

Pulmonary Hypertension Prognosis

A diagnosis of pulmonary hypertension (PH) is different for every person. Because there are several types and causes of PH, finding the right treatment may take time. Your healthcare team will consider your specific type of PH, symptoms, test results, and overall health when developing a treatment plan.

Treatment for PH can be complex, and regular follow-up is important. Over time, your doctor may adjust or change your treatment if your condition changes or if a therapy is no longer working as well as it should. Ongoing monitoring helps your healthcare team understand how you are responding to treatment and determine whether changes are needed.

There is currently no cure for most forms of PH, but treatment has made significant progress. Early diagnosis and appropriate treatment can help manage symptoms, improve quality of life, and, for many people, slow the progression of the disease.

PH can affect people of all ages, races, and ethnic backgrounds. The outlook varies widely depending on the type of PH and when it is diagnosed. Some forms of PH can be effectively treated or even potentially cured when the underlying cause is addressed, while other forms require lifelong treatment and monitoring.

Your prognosis is personal. Your PH specialist can help you understand what your diagnosis means for you, what to expect from treatment, and how your condition can be monitored over time.

Hope Through Research

Research continues to improve our understanding of pulmonary hypertension and lead to new treatment options. Clinical trials are an important part of this progress and offer patients opportunities to participate in research that may help advance care for people living with PH.