Pulmonary Hypertension: An Overview

Featured in the March, 2013 Scleroderma, Vasculitis & Myositis eNewsletter

What is pulmonary hypertension?

Pulmonary hypertension is a disease in which there is an increase in pressure in the blood vessel that connects the heart to the lungs, called the pulmonary artery. Because of this increase in pressure, the heart has to work harder in order to pump blood to the lungs.

What causes pulmonary hypertension?

There are many causes of pulmonary hypertension.

Connective tissue diseases can be considered a cause of pulmonary hypertension by causing inflammation or thickening, also known as fibrosis, of the pulmonary artery. These diseases include:

Lung diseases can also cause pulmonary hypertension by causing the pulmonary blood vessels to narrow, or constrict. These include:

  • Emphysema
  • Interstitial lung disease
  • Blood clots in the lung

Some forms of pulmonary hypertension are inherited and caused by a genetic mutation that can run in a family. Some patients can develop “idiopathic” pulmonary hypertension, which means the causes are unknown.

What are the symptoms of pulmonary hypertension?

There are many potential symptoms of pulmonary hypertension. These include:

  • Shortness of breath: Patients may notice that they are unable to walk as far as they used to, or that they are becoming short of breath when walking. They may notice that they become short of breath more easily when climbing stairs. In advanced stages of pulmonary hypertension, patients may be short of breath at rest.
  • Fatigue or exhaustion
  • Chest pain
  • Swelling of the legs
  • Fainting, or a feeling that you are about to faint
  • Dry cough

What should I do if I think I might have pulmonary hypertension?

Rheumatologists commonly encounter patients with pulmonary hypertension in their practices.

If you are concerned that you may have pulmonary hypertension, talk to your rheumatologist. He or she can perform the necessary history and physical exam, and refer you to a pulmonary hypertension specialist if necessary. Doctors who specialize in the treatment of pulmonary hypertension tend to be cardiologists and pulmonologists, although there are some rheumatologists who have a particular interest in the care of patients with pulmonary hypertension as well.

How common is pulmonary hypertension in patients with scleroderma?

Studies have shown that about 8 to 12% of patients with scleroderma develop some degree of pulmonary hypertension.

What can you expect from a doctor's visit for suspected pulmonary hypertension?

Your doctor will perform a complete history and physical exam. He or she will likely order some blood tests as well as one or more of the following diagnostic tests:

  • Electrocardiogram (EKG): This test measures the electrical activity of the heart.
  • Echocardiogram (Echo): This is an ultrasound of the heart that allows the doctor to see how well the heart is pumping and can estimate the pressure in the pulmonary artery.
  • Pulmonary function test (PFT): This test measures how much air your lungs can hold and how well you are able to breathe in and out.
  • Chest X-ray: This test allows your doctor to see if your heart is enlarged, if the blood vessel that connects your heart to your lungs is enlarged, and whether there is any scarring in your lungs.
  • Chest CT: This test allows your doctor to look for inflammation or scarring by providing a very clear picture of your lungs.
  • 6 minute walk test: This test measures how far you can walk in 6 minutes while also monitoring the oxygen level in your blood.
  • Right heart catheterization (right heart cath): This is the "gold standard" for diagnosing pulmonary hypertension. During this test, a thin tube is inserted into the right side of the heart to measure pressure. This test allows your doctor to determine which medications would be most appropriate for you.

Are there treatments for pulmonary hypertension?

Yes, there are several different medications for the treatment of pulmonary hypertension. Your doctor will determine what medication or combination of medications is right for you.

Are there any studies of pulmonary hypertension ongoing at Hospital for Special Surgery?

Yes. If you have scleroderma and have undergone a right heart catheterization within the prior 2 years, you may be eligible for a study of a 3-minute step test that evaluates how well your lungs work. If you are interested in participating, please contact Dr. Elana Bernstein at 212.774.2788 for more information.


Elana J. Bernstein, M.D.
Rheumatology Fellow, Hospital for Special Surgery

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