What is Pulmonary Hypertension (PH)?

Pulmonary hypertension (PH) is a condition where the blood pressure in the arteries of the lungs is higher than it should be. These arteries become narrowed, stiff, or blocked. As a result, the right side of the heart must work harder to push blood into the lungs, which can lead to symptoms such as shortness of breath, fatigue, and swelling.

PH is not the same as regular high blood pressure (hypertension).
It specifically involves the lungs and the right side of the heart.

Both patients and caregivers may find PH confusing at first — but understanding the basics can help you feel more supported and prepared for conversations with your care team.

Common Symptoms of PH

Many people with PH notice changes slowly over time. Symptoms may include:

Shortness of breath, especially during activity
Feeling unusually tired
Dizziness or lightheadedness
Heart racing or fluttering (palpitations)
Swelling in the legs, ankles, or abdomen
Chest pressure or discomfort

These symptoms can look like other conditions, which is why getting the right tests and diagnosis is important.

Explore Symptoms and Warning Signs

The Five Types (Groups) of Pulmonary Hypertension

Experts classify PH into five groups, based on what causes the high pressure in the lungs. This grouping helps PH specialists choose the right testing and treatment.

Group 1: Pulmonary Arterial Hypertension (PAH)
In PAH, the arteries in the lungs become narrowed or stiff. This increases pressure and makes the right side of the heart work harder. PAH can occur with no clear cause, or it can be related to conditions such as connective tissue disease, congenital heart disease, certain medications, or other causes.

Group 2: PH From Left Heart Disease
This is the most common type of PH. When the left side of the heart isn’t pumping or relaxing properly, blood can “back up” into the lungs, causing increased pressure in the pulmonary arteries.

Group 3: PH from Chronic Lung Diseases and/or Low Oxygen
Lung diseases such as pulmonary fibrosis, COPD, or sleep apnea can make it harder for oxygen to reach the bloodstream and cause narrowing of the blood vessels in the lungs. Over time, low oxygen levels due to lung diseases or other causes like sleep apnea or living at high altitude can increase pressures in the lung’s blood vessels.

Group 4: Chronic Blood Clots (CTEPH)
Chronic thromboembolic pulmonary hypertension (CTEPH) happens when blood clots in the lungs do not fully dissolve. Over time, they cause blockages and scarring in the pulmonary arteries.

Good news: CTEPH is one type of PH that can sometimes be improved or even cured with surgery or special procedures.

Group 5: PH from Other or Unclear Causes
Some conditions — such as sarcoidosis, sickle cell disease, certain metabolic disorders, or removal of the spleen — can lead to PH through complex pathways that don’t fit neatly into the other groups.

LEARN MORE ABOUT DIAGNOSTIC TESTING

Why Understanding the Type of PH Matters

Each group of PH has different causes, treatments, and follow-up needs. Because treatments that help one type of PH may not help (and could even harm) another type, it is essential to:

Get a full evaluation
Work with a PH specialist
Undergo the right diagnostic tests

Caregivers can play an important role in helping track symptoms, organize appointments, and support decision-making.

Learn How PH Is Diagnosed

When to Talk with a PH Specialist

If you’ve been told you might have PH — or if you have symptoms that don’t improve — a PH specialty center can provide the right tests and expertise. This may include:

Echocardiogram
CT scan or V/Q scan
Breathing tests
Blood tests
Right heart catheterization, the only test that can confirm PH and measure how severe it is

Getting the correct diagnosis early can make a meaningful difference in treatment and quality of life.

What is a PH Specialist?

A pulmonary hypertension (PH) specialist is a healthcare professional with advanced experience and expertise in diagnosing and managing all forms of pulmonary hypertension. PH specialists understand the complexities of this rare condition and are trained to interpret specialized tests, guide evidence-based treatment, and provide comprehensive, long-term care. PH specialists may be:

Pulmonologists (lung doctors)
Cardiologists (heart doctors)
Nurse Practitioners (NPs) or Physician Assistants (PAs) who work in dedicated PH programs

These clinicians typically practice in specialized PH centers, where multidisciplinary teams collaborate to evaluate symptoms, perform advanced diagnostics (such as right heart catheterization), monitor response to therapy, and tailor treatment plans to each patient’s needs.

A PH specialist’s goal is to ensure accurate diagnosis, provide access to the most effective therapies, and support patients in living healthier, fuller lives with pulmonary hypertension.