Pulmonary hypertension (PH) is high blood pressure in the blood vessels that carry blood from the right side of your heart to your lungs to pick up oxygen. When these blood vessels in the lungs become stiff, narrow, or damaged, it’s harder for blood to flow through. This raises the pressure in the lungs and makes your heart work harder.

Pulmonary hypertension isn’t the same as systemic hypertension — regular high blood pressure that affects the whole body and can damage organs and blood vessels over time. PH involves only the blood flow between your heart and lungs.

Although PH is mostly considered a lung disease, it can lead to serious heart issues, and some types of heart disease can cause PH. PH is a serious and progressive condition that can cause debilitating symptoms and complications like heart failure.

What’s the Main Cause of Pulmonary Hypertension?

PH can have many causes, and some cases are idiopathic, which means the cause is unknown. Common causes include:

• Lung disease such as chronic obstructive pulmonary disease (COPD)
• Heart problems, especially those affecting the left side of the heart
• Blood clots in the lungs
• Side effects of certain drugs or exposure to toxins
• Autoimmune diseases like scleroderma or lupus

• Inherited gene changes (called heritable pulmonary arterial hypertension)

Some people develop pulmonary arterial hypertension (PAH), a specific type of PH. PAH happens when the arteries in the lungs become narrow and stiff, raising pressure in those arteries.

Symptoms of Pulmonary Hypertension

Pulmonary hypertension might not cause any symptoms at first. Over time, you may notice:

• Shortness of breath, especially during everyday activities like walking or climbing stairs
• Fatigue (extreme tiredness that doesn’t improve with rest)
• Chest pain
• Dizziness or fainting
• Swelling in the legs, ankles, or belly

Diagnosis of Pulmonary Hypertension

Doctors who specialize in PH, such as pulmonologists (lung doctors), cardiologists (heart doctors), and rheumatologists (autoimmune specialists), work together to find the cause and best treatment plan.

Diagnosing PH often starts with a medical history and physical exam. The process may require multiple tests, including:

• An echocardiogram (ultrasound of the heart)
• Right heart catheterization (a test that measures pressure in the lung arteries and confirms PH)
• Blood tests
• Chest X-rays or lung scans

Your doctor will check how serious your symptoms are and how much they affect your daily life. People with PH are put into one of four groups, or classes. Class 1 means no symptoms, and class 4 means very severe symptoms, even at rest.

How Common Is Pulmonary Hypertension?

Researchers estimate that between 50 million and 70 million people worldwide are living with PH. The condition is often undiagnosed or mistaken for other illnesses.

PH usually develops between ages 30 and 60. Although the risk increases with age, PH can occur at any time and can even affect children. The Centers for Disease Control and Prevention (CDC) reports that PH is most common in women, non-Hispanic Black individuals, and people over the age of 75.

Can Pulmonary Hypertension Be Cured?

Although most types of pulmonary hypertension can’t be cured, PH can often be managed with treatment.

Medication, lifestyle changes, and sometimes procedures can help control symptoms, improve quality of life, and slow the progression of the condition. Your treatment will depend on the cause of your PH and may focus on treating your underlying conditions.

One type of PH, called chronic thromboembolic pulmonary hypertension (CTEPH), may be fixed with surgery.

Early diagnosis and working closely with your healthcare team are important for finding the best treatment plan for you. Continued care can make a significant difference in how you feel and live with pulmonary hypertension.

Outlook for Pulmonary Hypertension

Life expectancy, or how long someone lives with PH, depends on several factors, including the type of PH, how advanced it is, and whether other health problems are present. People who are diagnosed early and treated by a PH care team often live many years and maintain a good quality of life.

A Look Back: The History of Pulmonary Hypertension

Doctors first described PH in 1891, but its cause was a mystery. In 1929, a doctor named Werner Forssmann — who later, with his colleagues, received a Nobel Prize — developed a test called cardiac catheterization, which allowed doctors to measure pressure in the heart and lungs. Today, cardiac catheterization is the gold standard for diagnosing PH.

In the 1950s, researchers began linking PH to conditions like pulmonary embolisms (blood clots in the lungs) and low oxygen levels, such as at high altitudes. PH cases spiked in the 1960s and 1970s, especially in people taking certain diet pills. This led to more research and the creation of a patient registry in the U.S.

In 1995, the U.S. Food and Drug Administration (FDA) approved the first treatment for PH, a medication called epoprostenol (Flolan). Since then, many new medications and procedures have become available, and research into better treatments continues today.

Talk With Others Who Understand

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 others who understand life with pulmonary hypertension.

Have you or someone you love been diagnosed with pulmonary hypertension? Share your experience in the comments below, or start a conversation by posting on your Activities page.