PH Basics

An important step in transitioning to adult care is understanding your PH. Read on for an overview of:

Understanding Pulmonary Hypertension

Pulmonary hypertension (PH) means abnormally high blood pressure inside the lungs. PH is a rare blood vessel disorder of the lungs, in which the pressure in the pulmonary arteries rises above normal levels and may become life threatening.

The right ventricle (lower right heart chamber) pumps blood through your pulmonary arteries into your lungs. When the pressures are abnormally high, the right ventricle has to work harder to pump against this pressure. Over time, the right ventricle may become tired and not function as well as it should. The right heart catheterization, echocardiogram and cardiac MRI that you may be asked to get help your medical team to determine the function of your right ventricle and how to best treat you with medications.

You may hear people talk about PAH. PAH stands for pulmonary arterial hypertension. Although there is a technical and physiologic difference in meaning between PH and PAH, most people, including medical providers, will use these acronyms interchangeably.

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Symptoms of Pulmonary Hypertension

Symptoms of PH may include:

  • Breathlessness
  • Fatigue
  • Dizziness
  • Chest pain
  • Fainting
  • Swelling of the arms, legs or abdomen due to water retention

Being Diagnosed with Pulmonary Hypertension

If your doctor thinks you might have pulmonary hypertension, he or she will suggest that you have some tests done at the hospital. These tests help the doctor know if you have PH and, later on, whether the medicines that you take are making your PH better.

Some of these tests are:

Six-Minute Walk: This test looks at how far you can walk in six minutes and how much oxygen is in your blood before, during and after the test. During this test, you will walk up and down a hallway for 6 minutes.

Echo-Cardiogram (ECHO): This is a special ultrasound machine that takes pictures of your heart. You will put on a hospital gown and lie on your back. The echo tech will place a little bit of gel on your chest and use a special piece of equipment to take pictures of your heart. These pictures will show your doctor how well your heart is pumping and estimate how high the blood pressure is in your pulmonary artery. This test can take a long time - it often takes 45 minutes to an hour.

EKG: This test shows doctors the rhythm of your heart and gives the doctor a rough measure of the thickness of the chambers of your heart. For this test, stickers are put on your chest that are connected to a computer. The computer makes pictures of your heart rhythm. This test only takes a few minutes.   

Cardiac Catheterization: This is a procedure in which your doctor uses a small plastic catheter to directly measure the blood pressure in your heart and lungs and to see how well your heart is working. The procedure takes several hours and you may need to spend the night in the hospital afterwards. A right heart cardiac catheterization is one of the most accurate and useful tests for diagnosing PH.

Types of Pulmonary Hypertension

Idiopathic PH “Idiopathic” means unexplained, or in other words, the doctors do not know what caused or is causing the disease. Sometimes you will hear medical providers use the term, “primary pulmonary hypertension” for idiopathic PH.

Familial PH If more than one person in your family has PH, then you are said to have familial PH. A gene has been identified and linked to the cause of PH. 

PH can also be associated with other diseases or illnesses including:

  • Congenital heart defects
  • Pulmonary diseases (i.e. chronic obstructive pulmonary disease, cystic fibrosis)
  • Rheumatologic diseases (i.e. juvenile rheumatoid arthritis, scleroderma, lupus)
  • Liver disease
  • Thyroid disease
  • Blood clotting disorders
  • HIV
  • Recreational drugs or toxins

Learn more about these associated conditions

You should know what type of PH you have. If you don’t know, please discuss this with your medical team, and they will review this with you.

Treatments for Pulmonary Hypertension

There are a number of treatments for PH.

These medications work in different ways, but all of them try to dilate (relax) the pulmonary arteries. Depending on the medication, you might take it in different ways – your doctor can tell you more about how these medicines can help you and how you take them.

Learn more about different treatments for PH

Additional Resources for Understanding Pulmonary Hypertension

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The National Organization for Rare Disorders (NORD) awarded PHA the Abbey S. Meyers Leadership Award in 2012 for outstanding service to PHA members in advocacy, education and other key areas.