Pulmonary Hypertension

Pulmonary hypertension (PH) is a disease where you have abnormally high blood pressure in the blood vessels of your lungs (pulmonary arteries).  In PH, the pulmonary arteries become narrowed, and can be scarred to the point of being closed.  PH is a serious illness, and can be life-threatening.

What’s the Difference between PH and PAH?

Pulmonary Hypertension (PH) is the general term used to describe high blood pressure in the pulmonary arteries.  There are many causes of PH.

Pulmonary Arterial Hypertension (PAH) is one category of PH.  PAH is due to disease in the pulmonary arteries, which are narrowed and can be scarred to the point of being closed.  PAH is an important cause of PH because it is often the most severe, and because many new medications are for patients with PAH.

The World Health Organization (WHO) has grouped Pulmonary Hypertension into five categories. 

WHO Group 1 is Pulmonary Arterial Hypertension (PAH). This includes:

  • PAH that has no known cause (Idiopathic)
  • PAH that is familial or genetically inherited (hereditary)
  • PAH associated with connective tissue disease, congenital heart disease, severe liver disease, HIV infection, or the use of certain diet medications

WHO Groups 2 to 5 PH are linked to other conditions or diseases.

WHO Group 2 is PH due to left-sided heart disease, such as heart failure or high blood pressure.

WHO Group 3 is PH due to lung conditions such as COPD (chronic obstructive pulmonary disease), interstitial lung disease, and sleep apnea. WHO Group 4 is PH related to multiple or recurrent blood clots (pulmonary embolism) in the lungs.

WHO Group 5 is PH related to other diseases or conditions. Examples include diseases such as sarcoidosis, rare diseases such as lymphangioleiomyomatosis (LAM) and mediastinal fibrosis. This type can also be due to tumours putting pressure on the pulmonary arteries.

Pulmonary Hypertension can occur at any age, but it typically affects people between 20 and 60 years of age.

Pulmonary Hypertension can occur for no known reason (Idiopathic), but there are also some factors that increase the risk of Pulmonary Hypertension:

  • history of a close relative having PH (inherited)
  • use of appetite-suppressant medications for weight loss
  • history of pulmonary embolism (blood clots in the lungs)
  • other medical conditions including lung disease (such as COPD and sleep apnea), heart disease (such as heart failure), as well as connective tissue diseases (such as scleroderma), and severe liver disease
  • Infection with HIV/AIDS or Hepatitis B or C

Some of the signs of Pulmonary Hypertension include:

  • shortness of breath (dyspnea)
  • tiredness
  • chest pain, a heaviness, pressure or tightness in the centre of the chest (angina)
  • feeling faint or lightheaded
  • rapid, forceful or irregular heartbeat (palpitations)
  • swelling of feet and ankles (and possibly swelling of belly/abdomen)

Your doctor will diagnose PH based on your symptoms, family history, physical examination, and medical laboratory tests. Your doctor will also test you for the possible diseases that cause PH.

The medical tests could include chest X-rays, blood tests, pulmonary function tests (including spirometry), electrocardiogram (EKG), and echocardiogram and a six-minute walk test to see how you respond to exercise.

If left untreated, PH can get worse and can often lead to death. There are now medications available to treat PH which work to open up the narrowed pulmonary arteries, reducing the pulmonary artery pressure (PAP) and the pulmonary vascular resistance (PVR). As a result, the heart is better able to pump blood to the lungs.

Surgery (removal of blood clots, lung or heart-lung transplant) can effectively cure PH in some people.

If you or a loved one has PH the best treatment will be based on many factors such as:

  • the type of PH
  • the cause and severity of the PH
  • the age and overall health of the patient
  • the presence of other medical conditions and medications

Where can I get more information and support for Pulmonary Hypertension?

LivingwithPH.ca (Dr. Sanjay Mehta):  This website provides in-depth patient information on Pulmonary Hypertension, as well as images and videos.

The Pulmonary Hypertension Association (PAH)-Canada is an organization established by PH patients and their caregivers to provide support and education to the PH patient community.  The website provides information as well as opportunities to connect online and in person with PH patients and their caregivers across Canada.

National Heart, Lung and Blood Institute, a website from the US Department of Health and Human services, offers links to reliable information on Pulmonary Hypertension.