Pulmonary Hypertension

 

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What is pulmonary hypertension?

Pulmonary hypertension (PH) is high blood pressure in the lungs. Although it’s sometimes called “the other high blood pressure,” it is different from the systemic blood pressure your doctor measures with a cuff.

When the vessels in the lungs become narrow, stiff or blocked, pressure builds up causing the heart to work harder to pump blood through the lungs. This stress can lead to failure of the chamber on the right side of the heart, and ultimately death.

PH is considered a rare disease, however experts say that estimating the number of people with the disease is difficult.

PH is often misdiagnosed because doctors are likely to consider more common illnesses such as asthma as the primary cause of symptoms. Misdiagnosis is a serious problem for people with PH as it can delay critical treatment that could extend life and improve quality of life.

If you have another condition that is associated with pulmonary hypertension, such as connective tissue disease, pulmonary embolism, HIV infection, liver disease, heart disease, COPD or a family history of PH, talk with your doctor as these conditions could signal PH is present.

What are the symptoms of pulmonary hypertension?

Since PH mimics asthma, symptoms sometimes include common asthma symptoms such as:

  • dry cough
  • shortness of breath
  • difficulty breathing 
  • loss of energy 

Other symptoms may include:

  • dizziness
  • chest pain
  • fainting
  • swelling of the legs
  • feet and hands (also called edema)
  • Raynaud’s phenomenon, in which the patient has chalky white or blue fingers or toes that may become numb in cold weather. Patients’ lips and complexion may also appear blue due to low oxygen in the bloodstream.

How is pulmonary hypertension diagnosed?

The doctor will review your family and medical history and perform some preliminary tests. Tests may include:

  • blood tests
  • chest x-rays
  • electrocardiogram (ECG)
  • pulmonary function tests
  • echocardiogram
  • exercise tolerance tests.

If the results of initial tests point to PH, you will likely be referred to a specialist. A right-heart catheterization and a ventilation/perfusion lung scan must be done to determine diagnosis, prognosis and treatment.

How is pulmonary hypertension treated?

At this time there is no cure for PH, but the last 25 years have seen significant advances in treatment options for people living with the disease – including 14 FDA-approved targeted medications for two forms of PH. Patients may also be treated with oxygen therapy.

For patients with a form of the disease caused by blood clots in the lungs, a highly specialized operation that removes blockages from arteries in the lungs may be an option.

If pulmonary hypertension is caused by certain medical conditions, heart disease or lung disease, additional treatments will focus on the underlying illness.

Patient Resources

Pulmonary Hypertension Association: PHAssociation.org 

PHA Classroom: PHAClassroom.org 

American Thoracic Society: thoracic.org

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