Pulmonary hypertension (PH) is a chronic and progressive disease associated with high blood pressure in the blood vessels that supply the lungs, known as the pulmonary arteries.

The right side of the heart pumps deoxygenated blood into the pulmonary artery. The pulmonary artery branches into smaller blood vessels in the lungs to remove carbon dioxide and pick up oxygen. The re-oxygenated blood passes through the left side of the heart before being transported throughout the body.

Types of pulmonary hypertension

The World Health Organization (WHO) created five groups to classify types of pulmonary hypertension, based on the underlying cause and symptoms of the disease.

  • Group 1, also called pulmonary arterial hypertension (PAH), is caused by a narrowing of the arteries. There are multiple sub-types: idiopathic (no known cause); inherited; due to drugs or toxins; and related to other conditions (such as HIV or congenital heart disease).
  • Group 2 includes PH caused by left heart disease. This is the most common type.
  • Group 3 is pulmonary hypertension due to lung diseases such as chronic obstructive pulmonary disease (COPD), pulmonary fibrosis, and sleep apnea.
  • Group 4 is PH caused by chronic blood clots in the lungs, also known as chronic thromboembolic pulmonary hypertension (CTEPH).
  • Group 5 is PH with an unclear cause occurring alongside other diseases. This can include blood disorders, metabolic disorders, sarcoidosis, or tumors.

Symptoms of PH

The main symptoms associated with pulmonary hypertension are shortness of breath, fatigue, dizziness or fainting, chest pain, swelling of the ankles, legs, and abdomen, blue-tinted lips and skin, and heart palpitations (a racing heartbeat).

These symptoms can make it difficult to exercise, including in routine tasks such as climbing stairs.

The increased blood pressure in the lungs means that the right side of the heart must work harder to pump blood through the arteries. This can lead to heart failure in later life.

Treating pulmonary hypertension

There is currently no cure for PH, but there are medications available to manage the symptoms of the disease.

It is important that in pulmonary hypertension associated with another condition, the underlying condition is treated first, as this can slow the progression of PH.

The treatment will depend on the type of pulmonary hypertension. For example, patients with group 1, or PAH, may be prescribed medications to widen the blood vessels, such as Adcirca (tadalafil) or Letairis (ambrisentan).

Some patients may be recommended for surgical procedures, such as a lung transplant, if the underlying cause of their PH is lung disease.

Other common treatments include:

  • Diuretic medications, to manage swelling by reducing fluid build-up;
  • Digoxin, to strengthen the heart muscles so that blood can be pumped more effectively;
  • Oxygen therapy, or supplementary oxygen, where additional oxygen is delivered through a face mask or tubes to reduce tiredness and breathlessness;
  • Blood-thinning medications (also called anticoagulants), to prevent blood clot formation in the arteries, or to prevent existing clots from increasing in size.


Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.