Pulmonary Hypertension Types, Symptoms, and Treatment

High Blood Pressure in the Pulmonary Arteries

Pulmonary hypertension
Pulmonary hypertension. Photo © A.D.A.M.

The heart pumps blood to the lungs to get oxygen through blood vessels called pulmonary arteries. Sometimes the pressure in these arteries increases above normal. This is called pulmonary hypertension (PH).

People of any age, from newborn babies to the elderly, can develop pulmonary hypertension, although it usually develops between the ages of 20 and 60. It may be caused by certain diseases or conditions, be inherited, or have an unknown cause.

Pulmonary hypertension develops when changes happen in the cells of the pulmonary arteries. The walls of the arteries tighten and blood clots form. The walls of the arteries are stiff either from birth or from the overgrowth of cells over time. These changes mean that the pulmonary arteries can’t carry as much blood, and it is harder for the heart to pump blood through them.

Types of Pulmonary Hypertension

The World Health Organization divides pulmonary hypertension into five groups, based on cause.

  • Group 1, called pulmonary arterial hypertension (PAH), includes inherited PAH, PAH that has no known cause, and PAH with causes such as congenital heart disease, HIV infection, the use of certain diet medicines or street drugs (such as cocaine), and thyroid diseases. Group 1 PAH that has no known cause may be called primary or idiopathic PAH.
  • Group 2 includes pulmonary hypertension with heart disease on the left side of the heart, such as mitral valve disease.
  • Group 3 includes pulmonary hypertension with lung conditions such as COPD and interstitial lung disease. This group also includes sleep disorders that cause breathing problems, such as sleep apnea.
  • Group 4 includes pulmonary hypertension due to blood clots in the lungs, blood clotting disorders, or sickle cell anemia.

When pulmonary hypertension occurs with or is caused by another disease or condition, it may be called secondary pulmonary hypertension.

Symptoms of Pulmonary Hypertension

The symptoms of pulmonary hypertension may include:

  • tiredness
  • shortness of breath during routine activities, such as climbing two flights of stairs
  • chest pain
  • a fast heartbeat
  • feeling lightheaded during activity
  • swelling in the legs and ankles
  • a bluish color (cyanosis) on lips and skin
  • fainting

In early pulmonary hypertension, there may be no symptoms. As the disease worsens, its symptoms may limit physical activity.

Diagnosis of Pulmonary Hypertension

Diagnosis of pulmonary hypertension is based on medical history, physical examination, and medical tests. In taking a medical history, the doctor asks about symptoms, any other medical conditions the person has, and if any family members have pulmonary hypertension.

An important medical test that helps detect pulmonary hypertension is an ultrasound of the heart (echocardiogram). This test gives the doctor a picture of the heart and the pulmonary arteries, and can also estimate the pressure in the arteries. A chest x-ray can show whether the heart and pulmonary arteries are enlarged, and may show if any lung disease is present.

A special test called heart catheterization may be done, too. In this test, a long, thin, flexible tube called a catheter is inserted in a blood vessel in the groin and threaded up to the heart. It can measure the pressure in the pulmonary arteries and show how well the heart is working.

Other medical tests may be done to look for possible causes of pulmonary hypertension. An exercise test will be used to find out how severe the pulmonary hypertension is.

Treatment of Pulmonary Hypertension

Treatment of pulmonary hypertension may help relieve the symptoms and slow the progress of the disease. Treatment depends on what type of PH is present. If secondary pulmonary hypertension, treating or curing the underlying disease may make the PH go away.

Some common treatments for pulmonary hypertension are:

  • digoxin, a medication that helps the heart pump blood more efficiently
  • blood-thinning medicines to help prevent blood clots
  • diuretics (“water pills”) to help reduce swelling in the legs and ankles
  • oxygen
  • lifestyle changes, such as quitting smoking, maintaining a healthy weight, and having regular physical exercise

In some cases, medical procedures or surgery may be needed to treat pulmonary hypertension or an underlying disease such as lung disease.


"Pulmonary Hypertension." Lung Diseases. Sep 2008. National Heart Lung and Blood Institute. 18 Nov 2008

"Pulmonary Hypertension." Associated Conditions. American Heart Association. 18 Nov 2008

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